BIOMARIN PHARMACEUTICAL INC
DEF 14A, 2000-04-28
PHARMACEUTICAL PREPARATIONS
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                            SCHEDULE 14A INFORMATION

                PROXY STATEMENT PURSUANT TO SECTION 14(a) OF THE

                         SECURITIES EXCHANGE ACT OF 1934
                                (AMENDMENT NO. )

Filed by the Registrant  [X]

Filed by a Party other than the Registrant [  ]

Check the appropriate box:

[  ]  Preliminary Proxy Statement

[  ]  Confidential, for Use of the Commission Only (as permitted by
        Rule 14a-6(e) (2))
[X ]  Definitive Proxy Statement
[  ]  Definitive Additional Materials

[  ]  Soliciting Material Pursuant to 240.14a-11(C)or 240.14a-12

                          BioMarin Pharmaceutical Inc.

- --------------------------------------------------------------------------------
                (Name of Registrant as Specified In Its Charter)

- --------------------------------------------------------------------------------
    (Name of Person(s) Filing Proxy Statement, if other than the Registrant)

Payment of Filing Fee (Check the appropriate box):

[X]  No fee required.

[ ] Fee computed on table below per Exchange Act Rules 14a-6(i) (4) and 0-11.

(1)      Title of each class of securities to which transaction applies:

- --------------------------------------------------------------------------------
(2)      Aggregate number of securities to which transaction applies:

- --------------------------------------------------------------------------------
(3)           Per unit price or other underlying  value of transaction  computed
              pursuant to Exchange  Act Rule 0-11 (Set forth the amount on which
              the filing fee is calculated and state how it was determined):

- --------------------------------------------------------------------------------
(4)      Proposed maximum aggregate value of transaction:

- --------------------------------------------------------------------------------
(5)      Total fee paid:
- --------------------------------------------------------------------------------

[ ] Fee paid previously with preliminary materials.

[ ] Check box if any part of the fee is offset as provided by exchange  Act
       Rule 0-11 (a) (2) and  identify the filing for which the  offsetting fee
       was paid previously.  Identify the previous  filing by registration
       statement number, or the Form or Schedule and the date of its filing.

(1)      Amount Previously Paid:
- --------------------------------------------------------------------------------
(2)      Form, Schedule or Registration Statement No.:
- --------------------------------------------------------------------------------
(3)      Filing Party:
- --------------------------------------------------------------------------------
(4)      Date Filed:
- --------------------------------------------------------------------------------
<PAGE>
                          BioMarin Pharmaceutical Inc.

                     371 Bel Marin Keys Boulevard, Suite 210

                                Novato, CA 94949

                    NOTICE OF ANNUAL MEETING OF STOCKHOLDERS

                           TO BE HELD ON JUNE 15, 2000

TO THE STOCKHOLDERS:

         NOTICE IS HEREBY  GIVEN that the 2000  Annual  Meeting of  Stockholders
(the  "Annual  Meeting")  of BioMarin  Pharmaceutical  Inc.  ("BioMarin"  or the
"Company") will be held on Thursday, June 15, 2000 at 10:00 A.M., local time, at
the Company's facility located at 46 Galli Drive,  Novato,  California 94949 for
the following purposes:

         1.       To elect five directors of the Company;

         2.       To ratify the selection by the Board of Directors of Arthur
                  Andersen LLP as the Company's  independent  auditors for
                  the year ending December 31, 2000;

         3.       To transact such other business as properly may be brought
                  before the Annual Meeting or any adjournment thereof.

         The foregoing  items of business are more fully  described in the proxy
statement accompanying this notice (the "Proxy").

         The Board of  Directors  has fixed the close of  business  on April 17,
2000 as the record date for  determining  the  stockholders  entitled to receive
notice  of, and to vote at, the Annual  Meeting or any  adjournment  thereof.  A
complete list of such stockholders will be available at the Company's  executive
offices at 371 Bel Marin Keys Boulevard,  Suite 210, Novato,  California  94949,
for ten days before the Annual Meeting.

         All stockholders are cordially invited to attend the Annual Meeting. To
ensure your  representation  at the Annual  Meeting,  however,  you are urged to
complete,  date,  sign and return the enclosed Proxy as promptly as possible.  A
postage-prepaid envelope is enclosed for that purpose. Any stockholder attending
the Annual  Meeting may vote in person even if that  stockholder  has returned a
Proxy.

By Order of the Board of Directors
/s/ Raymond W. Anderson
Raymond W. Anderson

Chief Financial Officer and Vice President, Finance and Administration

Novato, California
April 20, 2000

                             YOUR VOTE IS IMPORTANT

IN ORDER TO ASSURE YOUR REPRESENTATION AT THE ANNUAL MEETING,  YOU ARE REQUESTED
TO COMPLETE, SIGN AND DATE THE ENCLOSED PROXY AS PROMPTLY AS POSSIBLE AND RETURN
IT IN THE ENCLOSED ENVELOPE.






                          BioMarin Pharmaceutical Inc.

                     371 Bel Marin Keys Boulevard, Suite 210

                                Novato, CA 94949

                               PROXY STATEMENT FOR

                       2000 ANNUAL MEETING OF STOCKHOLDERS

INFORMATION CONCERNING SOLICITATION OF PROXIES AND VOTING

General

This Proxy is furnished in connection  with the  solicitation  of Proxies by the
Board of Directors of the Company for use at the Annual Meeting of  Stockholders
of the Company (the "Annual Meeting") to be held on Thursday,  June 15, 2000, or
at any adjournment of the Annual Meeting,  for the purposes set forth herein and
in the foregoing  Notice of Annual Meeting of  Stockholders.  The Annual Meeting
will be held at the  Company's  facilities  located at 46 Galli  Drive,  Novato,
California  94949.  The phone  number is  415-884-6700.  Copies of  solicitation
material and the Company's Annual Report including the financial  statements and
financial statement schedules will be furnished to brokerage houses, fiduciaries
and  custodians to forward to  beneficial  owners of common stock of the Company
held in their names. The cost of solicitation of Proxies,  including expenses in
connection with preparing and mailing this Proxy,  will be borne by the Company.
In  addition,  the Company  will  reimburse  brokerage  firms and other  persons
representing  beneficial owners of common stock for their expenses in forwarding
solicitation  material  to such  beneficial  owners.  Original  solicitation  of
Proxies by mail may be supplemented by telephone,  facsimile,  e-mail,  telegram
and personal  solicitation by directors,  officers or other regular employees of
the Company. No additional  compensation will be paid to directors,  officers or
other regular employee for such services.  The Annual Report to Stockholders for
the fiscal year ended December 31, 1999, including financial statements, will be
mailed to stockholders  entitled to vote at the Annual Meeting concurrently with
this Proxy Statement and accompanying Proxy on or about May 16, 2000.

Record Date; Outstanding Securities

The voting  securities  of the Company  entitled  to vote at the Annual  Meeting
consist of shares of common stock.  Only  stockholders of record at the close of
business on April 17,  2000 are  entitled to notice of and to vote at the Annual
Meeting.  On April 17, 2000,  there were 35,321,721  shares of common stock, par
value $0.001 per share,  issued and  outstanding.  Each share of common stock is
entitled  to one vote.  As of the date of  record,  no  shares of the  Company's
Preferred Stock were outstanding.

Revocability of Proxies

A stockholder  who signs and returns a Proxy will have the power to revoke it at
any time  before it is voted.  A Proxy may be revoked by filing with the Company
(Attention:  Raymond W. Anderson, Chief Financial Officer) a written revocation,
or a duly  executed  Proxy  bearing a later date,  or by appearing at the Annual
Meeting and electing to vote in person.
<PAGE>
Voting

Each stockholder is entitled to one vote for each share held.

Solicitation of Proxies

This solicitation of Proxies is made by the Company,  and all related costs will
be borne by the Company. In addition, the Company will reimburse brokerage firms
and other persons representing beneficial owners of shares for their expenses in
forwarding  solicitation material to such beneficial owners. Proxies may also be
solicited by certain of the Company's directors, officers and regular employees,
without additional  compensation,  in person or by telephone or by facsimile, or
by e-mail or by telegram.

Quorums; Abstentions; Broker Non-Votes

The  Company's  Bylaws  provide  that a majority of all the shares of the common
stock entitled to vote,  whether present in person or by Proxy, shall constitute
a quorum for the transaction of business at the Annual  Meeting.  If a quorum is
not present or  represented,  then either the chairman of the Annual  Meeting or
the  stockholders  entitled to vote at the Annual Meeting,  present in person or
represented  by Proxy,  will have the power to adjourn the Annual  Meeting  from
time to time,  without notice other than an  announcement at the Annual Meeting,
until a quorum is present.  At any adjourned Annual Meeting at which a quorum is
present,  any business may be transacted  that might have been transacted at the
Annual  Meeting as  originally  notified.  If the  adjournment  is for more than
thirty  days,  or if after that  adjournment  a new record date is fixed for the
adjourned  Annual  Meeting,  a notice of the adjourned  Annual  Meeting shall be
given to each  stockholder  of record  entitled to vote at the adjourned  Annual
Meeting.  If an executed  Proxy is  submitted  without any  instruction  for the
voting  of such  Proxy,  the  Proxy  will be voted  in  favor  of the  proposals
described.

All shares  represented  by valid Proxies  received  prior to the Annual Meeting
will be voted and, where a stockholder  specifies by means of the Proxy a choice
with  respect  to any  matter  to be acted  upon,  the  shares  will be voted in
accordance with the specification so made.

Votes cast by Proxy or in person at the Annual  Meeting will be tabulated by the
Inspector  of  Elections  (the  "Inspector")  who  will  be an  employee  of the
Company's  transfer  agent.  The Inspector will also determine  whether or not a
quorum is present.  Except in certain  specific  circumstances,  the affirmative
vote of a majority of shares present in person or represented by Proxy at a duly
held Annual  Meeting at which a quorum is present is required under Delaware law
for approval of proposals  presented to stockholders.  In general,  Delaware law
also  provides that a quorum  consists of a majority of shares  entitled to vote
that are present or represented by Proxy at the Annual Meeting.

The Inspector will treat shares that are voted  "WITHHELD" or "ABSTAIN" as being
present and  entitled  to vote for  purposes of  determining  the  presence of a
quorum but such  shares will not be treated as votes in favor of  approving  any
matter  submitted  to the  stockholders  for a vote.  When  Proxies are properly
<PAGE>
dated,  executed and returned,  the shares  represented  by such Proxies will be
voted  at  the  Annual  Meeting  in  accordance  with  the  instructions  of the
stockholder. If no specific instructions are given, the shares will be voted for
(i) the  election of the  nominees  for  directors  set forth  herein;  (ii) the
ratification of Arthur  Andersen LLP as independent  auditors of the Company for
the fiscal year ending December 31, 2000;  (iii) upon such other business as may
properly come before the Annual Meeting or any adjournment thereof.

If a broker  indicates on the enclosed proxy or its substitute  that such broker
does  not  have  discretionary  authority  as to  certain  shares  to  vote on a
particular matter ("Broker  Non-Votes"),  those shares will not be considered as
present with respect to that matter.  The Company  believes that the  tabulation
procedures  to be  followed by the  Inspector  are  consistent  with the general
statutory requirements in Delaware concerning voting of shares and determination
of a quorum.

Stockholders  who intend to submit a proposal  for  inclusion  in the  Company's
proxy  materials for the 2001 Annual  Meeting of  Stockholders,  must submit the
proposal to the Company no later than February 15, 2001. Stockholders who intend
to  present a  proposal  at the 2001  Annual  Meeting  of  Stockholders  without
inclusion of such proposal in the Company's  proxy materials for the 2001 Annual
Meeting are required to provide  notice of such proposal to the company no later
than March 31,  2001.  The  Company  reserves  the right to reject,  rule out of
order, or take other  appropriate  action with respect to any proposal that does
not comply with these and other applicable requirements.


<PAGE>



SECURITY OWNERSHIP OF CERTAIN BENEFICIAL OWNERS

The  following  table  sets  forth  certain  information  with  respect  to  the
beneficial  ownership  of our common  stock as of March 31,  2000 as to (i) each
person, or group of affiliated  persons,  who is known by us to own beneficially
more than 5% of the common stock ; (ii) each of our directors; (iii) each of our
Named  Executive  Officers;  (iv) all of our  directors  and  current  executive
officers as a group.

Except as  otherwise  noted,  the  persons or  entities  in this table have sole
voting  and  investing  power with  respect  to all the  shares of common  stock
beneficially owned by them subject to community property laws, where applicable.
<TABLE>

<CAPTION>
                                                      Number of             Number of          Percentage of
                                                       Shares                 Shares           Common Stock
                                                    Beneficially             Subject            Outstanding
           Name of Beneficial Owner                  Owned (1)             To Options
           ------------------------                  ----------            ----------          ------------

<S>                                                  <C>                   <C>                 <C>
Glyko Biomedical Ltd...........................      11,367,617                  0                  32.2%
   371 Bel Marin Keys Blvd., Suite 210
   Novato, CA 94949


BB BioVentures, L.P.(2)........................       2,725,787                  0                  7.7%
   One Cambridge Center, 9th Floor
   Cambridge, MA 02142


Genzyme Corporation............................       2,102,563                  0                  6.0%
   One Kendall Square
   Cambridge, MA 02139


Grant W. Denison, Jr...........................       1,546,127               246,127               4.4%


John C. Klock, M.D.(3).........................      12,426,337               305,720               34.9%


<PAGE>




                                                      Number of             Number of       Percentage of Common
                                                       Shares                 Shares                Stock
                                                    Beneficially             Subject             Outstanding
           Name of Beneficial Owner                  Owned (1)             To Options
           ------------------------                  ----------            ----------       ---------------------


Christopher M. Starr, Ph.D.....................        579,094                194,094               1.6%


Emil D. Kakkis, M.D., Ph.D.....................        109,099                107,099                 *


Raymond W. Anderson............................        137,857                137,857                 *


Ansbert S. Gadicke, M.D., Ph.D.(4).............       2,784,537                58,750               7.9%


Erich Sager(5).................................       1,313,500                62,500               3.7%


Gwynn R. Williams(6)...........................      11,410,117                42,500               32.3%


All current executive officers and directors
as a group(10 persons)(7)......................      19,059,809              1,275,405              52.1%
<FN>

* Represents less than 1% of the Company's outstanding common stock.

(1)  The  "Number  of  Shares  Beneficially  Owned"  column  below  is  based on
     35,305,921  shares of common stock outstanding at March 31, 2000. Shares of
     common stock subject to options or warrants that are currently  exercisable
     or  exercisable  within 60 days of March 31,  2000 (the  "Number  of Shares
     Beneficially  Owned") are deemed to be outstanding  and to be  beneficially
     owned by the person  holding  the  options or  warrants  for the purpose of
     computing  the  percentage  ownership  of the person but are not treated as
     outstanding  for the purpose of computing the  percentage  ownership of any
     other person. The "Number of Shares Subject to Options" enumerates for each
     principal  stockholder,  director and Named  Executive  Officer and for all
     officers and directors in aggregate,  the shares of common stock subject to
     options  exercisable  within 60 days of March 31,  2000.  These  shares are
     included in the calculation of the "Number of Shares Beneficially Owned."
<PAGE>
 (2) Includes shares held by MPM Asset Management 1998 Investors L.L.C. and MPM
       BioVentures Parallel Fund L.P., both of which are affiliated with BB
       BioVentures L.P.

 (3) Includes 11,367,617 shares held by Glyko Biomedical Ltd. of which Dr. Klock
     is an officer, director and stockholder. Dr. Klock disclaims beneficial
     ownership of these shares, except to the extent of his pecuniary interest.

 (4) Includes 2,725,787 shares currently held by BB BioVentures L.P., MPM
      Asset Management 1998 Investors L.L.C. and MPM BioVenture Parallel Fund
      L.P., of which Dr. Gadicke is an affiliate. Dr. Gadicke disclaims
      beneficial ownership in these shares except to the extent of his pecuniary
      interest.

 (5) Includes 1,221,000 shares of common stock currently held by LaMont Asset
      Management SA, and 60,000 shares held by Belmont Capital Ltd. S.A.
      Mr. Sager is an affiliate of each entity. Mr. Sager disclaims beneficial
      ownership of the shares of LaMont Asset Management SA and Belmont Capital
      Ltd., except to the extent of his pecuniary interest in each entity.

 (6) Includes 11,367,617 shares of common stock currently held by Glyko
      Biomedical Ltd., of which Mr. Williams is a director and a stockholder.
      Mr. Williams disclaims beneficial ownership of these shares except to the
      extent of his pecuniary interest.

 (7)  See footnotes 2 through 5.
</FN>
</TABLE>

<PAGE>


PROPOSAL ONE:  ELECTION OF DIRECTORS

The Company has a Board of Directors,  currently  consisting of five  directors,
which will be elected at the Annual Meeting.  The Proxy holders may not vote the
Proxies  for a greater  number of  persons  than the number of  nominees  named.
Unless otherwise instructed, the Proxy holders will vote the Proxies received by
them for the five nominees named below,  all of whom are presently  directors of
the Company.  If any nominee is unable or declines to serve as a director at the
time of the Annual Meeting, the Proxies will be voted for any nominees who shall
be designated  by the present Board of Directors to fill the vacancy.  It is not
expected  that any  nominee  will be  unable  to or will  decline  to serve as a
Director. If stockholders nominate additional persons for election as directors,
the Proxy holder will vote all Proxies received by him to assure the election of
as many of the Board of  Directors'  nominees as possible  with the Proxy holder
making any required  selection of specific nominees to be voted for. The term of
office of each person elected as a director shall continue until the next Annual
Meeting of Stockholders or until that person's successor has been elected.

If a quorum is present  and  voting,  the five  nominees  receiving  the highest
number of affirmative votes of the votes cast shall be elected as directors.

NOMINEES FOR DIRECTOR

Set forth below is certain information regarding our directors:
<TABLE>
<CAPTION>
          Name                Age         Position with BioMarin          Director Since
          ----                ---         ----------------------          --------------
<S>                           <C>   <C>                                   <C>
Grant W. Denison, Jr.          49   Chief Executive Officer and
                                     Chairman of the Board                 October 1996
John C. Klock, M.D.            54   President, Secretary and Director      October 1996
Ansbert S. Gadicke, M.D.,
 Ph.D. (1)(2)                  41   Director                               December 1997
Erich Sager(1)(2)              41   Director                               November 1997
Gwynn R. Williams(1)(2)        65   Director                               October 1996
<FN>

    (1)           Member of the Compensation Committee.
    (2)           Member of the Audit Committee.
</FN>
</TABLE>

There is no family  relationship  between any director and any executive officer
of the Company.

Grant W. Denison,  Jr. has served as a director and Chief  Executive  Officer of
the Company  since its  inception and as Chairman of the Board since April 1997.
From July 1993 to April 1997, Mr. Denison served as President, Consumer Products
and  Corporate  Senior  Vice  President,   Business  Development  at  Searle,  a
pharmaceutical  company. From July 1989 to June 1993, Mr. Denison served as Vice
President  Corporate  Planning at Monsanto Company,  a diversified life sciences
company.  From April 1986 to June 1989,  Mr.  Denison  served as Vice  President
Corporate  Planning and President,  U.S.  Operations at Searle, a pharmaceutical
company. From 1985 to 1986, Mr. Denison served as Vice President,  International
Operations at Squibb Medical Systems,  a medical devices  company.  From 1980 to
<PAGE>
1985, Mr. Denison served as Vice President, Planning and Business Development at
Pfizer,  Inc., a  pharmaceutical  company.  Mr.  Denison serves as a director of
Nastech   Pharmaceutical   Company  Inc.,   Dentalview,   Inc.,   York  Medical,
BioNebraska,  Inc., Equity 4 Life and Clubb BioCapital.  Mr. Denison received an
A.B. in  Mathematical  Economics  from  Colgate  University  and an M.B.A.  from
Harvard Graduate School of Business Administration.

John C. Klock, M.D. has served as the President and Secretary of the Company and
served as a director since its inception.  Dr. Klock has served as the President
of Glyko,  Inc. since October 1989. Dr. Klock was a founder of Glyko  Biomedical
Ltd.  and its  predecessor  Glyko,  Inc.  and has served as a director  of Glyko
Biomedical  Ltd.  since  December  1992.  Dr.  Klock was a founder  of  Glycomed
Incorporated,  a  biotechnology  company,  at which he served as Vice President,
Medical Affairs from July 1987 to July 1990. Dr. Klock was a scientific director
at the Institute of Cancer  Research at California  Pacific  Medical Center from
July 1981 to July 1987.  Dr. Klock was an academic  physician  and  carbohydrate
researcher at the  University of California at San Francisco  from 1982 to 1986.
Dr.  Klock  received a B.S.  in Zoology  from  Louisiana  State  University  and
received an M.D. from Tulane University.

Ansbert S. Gadicke,  M.D.,  Ph.D., has served as a director of the Company since
December  1997.  Since July 1992,  Dr. Gadicke has served as the Chairman of the
Board  and  Managing  Director  of MPM  Capital,  L.P.,  an  investment  company
specializing  in the healthcare  industry.  From 1989 to 1992, Dr. Gadicke was a
consultant with Boston  Consulting  Group.  Dr. Gadicke  currently serves on the
boards of Coelacanth Corporation,  DoubleTwist, Inc., LXN Corporation,  MediGene
AG, Novirio  Pharmaceuticals  Limited, Omrix  Pharmaceuticals,  Inc., Pharmasset
Ltd., ViaCell, Inc. and Transform, Inc. Dr. Gadicke received a Ph.D. and an M.D.
from J.W. Goethe Universitat, Frankfurt, Germany.

Erich Sager has served as a director of the Company since November  1997.  Since
September 1996, Mr. Sager has served as the Chairman of LaMont Asset  Management
SA, a private  investment  management  firm. From April 1994 to August 1996, Mr.
Sager served as Senior Vice President, Head of Private Banking for Dresdner Bank
(Switzerland)  Ltd. From  September 1991 to March 1994, Mr. Sager served as Vice
President, Private Banking-Head German Desk for Deutsche Bank (Switzerland) Ltd.
From 1981 to 1989,  Mr.  Sager held  various  positions  at a number of banks in
Switzerland.  Mr.  Sager  serves as a director  of  BioNebraska,  Inc.,  Comptec
Industries Ltd.,  Dentalview,  Inc.,  LaMont Asset  Management,  SA, and Sermont
Asset  Management,  SA. Mr. Sager received a Business  Degree from the School of
Economics and Business Administration in Zurich, Switzerland.

Gwynn R. Williams has served as a director of the Company  since its  inception.
Mr. Williams founded AstroMed Limited and Astroscan Limited, UK manufacturers of
scientific  equipment,  in March 1984, which entities,  in December 1997, merged
into Life  Science  Resources  Ltd.  Previously,  Mr.  Williams was a partner in
Arthur  Andersen & Co., a  mathematician  with General  Motors  Research,  and a
mathematician with British Steel. Mr. Williams was a founder of Glyko Biomedical
Ltd. and its predecessor Glyko, Inc. Mr. Williams received a B.S. in Theoretical
Physics from the University of Wales.
<PAGE>
Board Meetings and Board Committees

The Board of Directors of the Company held a total of nine  meetings  during the
year ended December 31, 1999. No director  participated in fewer than 75% of all
such meetings and actions of the Board of Directors and the committees  thereof,
held during fiscal 1999, if any, upon which such director served.

The Board has established an Audit Committee and a Compensation  Committee.  The
Audit  Committee,  which  consisted  of Dr.  Gadicke  and Mr.  Williams in 1999,
reviews  the  Company's   consolidated   financial   statements  and  accounting
practices,  makes  recommendations  to the  Board  regarding  the  selection  of
independent  auditors  and  reviews the results and scope of the audit and other
services provided by the Company's independent auditors. The Audit Committee met
once in 1999. Mr. Sager joined the Audit Committee in 2000.

The  Compensation  Committee,  which consists of Dr. Gadicke,  Mr. Sager and Mr.
Williams,  sets  general  compensation  policy  for the  Company  and has  final
approval  power  over  compensation  of  executive  officers.  The  Compensation
Committee  also has final  approval  power  over  guidelines  and  criteria  for
employees'  bonuses and administers the 1997 Stock Plan and 1998 Director Option
Plan. The Compensation Committee met twice in 1999.

Compensation Committee Interlocks and Insider Participation

None of the members of the  Compensation  Committee is currently or has been, at
any time since the  formation  of the  Company,  an officer or  employee  of the
Company. No member of the Compensation Committee serves as a member of the Board
of  Directors  or  compensation  committee  of any  entity  that has one or more
executive  officers  serving as a member of the Company's  Board of Directors or
Compensation Committee.

Mr. Williams serves on the Board of Directors of Glyko  Biomedical Ltd. (GBL) of
which Dr. Klock and Mr. Anderson are also directors.

PROPOSAL TWO:  RATIFICATION OF APPOINTMENT OF INDEPENDENT AUDITORS

The Board of Directors has selected Arthur Andersen LLP,  independent  auditors,
to audit the  financial  statements  of the  Company  for the fiscal year ending
December 31, 2000, and recommends  that  stockholders  vote for  ratification of
such  appointment.  Although  action by stockholders is not required by law, the
Board of Directors has  determined  that it is desirable to request  approval of
this selection by the stockholders.  Notwithstanding the selection, the Board of
Directors,  in its  discretion,  may direct the  appointment of new  independent
auditors at any time during the year, if the Board of Directors  feels that such
a change would be in the best interest of the Company and its  stockholders.  In
the  event of a  negative  vote on  ratification,  the Board of  Directors  will
reconsider its selection.

Arthur  Andersen LLP has audited the  Company's  financial  statements  annually
since  inception on March 21, 1997.  Representatives  of Arthur Andersen LLP are
expected  to be present at the Annual  Meeting  with the  opportunity  to make a
statement if they desire to do so and are expected to be available to respond to
appropriate questions.
<PAGE>
The  Board   unanimously   recommends  voting  "For"  the  ratification  of  the
appointment of Arthur  Andersen LLP as  independent  auditors of the Company for
the fiscal year ending December 31, 2000.


<PAGE>



EXECUTIVE OFFICERS

In addition to Grant W. Denison,  Jr., the Company's Chief Executive Officer and
Chairman of the Board, and John C. Klock, M.D., the Company's  President and one
of its directors, whose biographies appear above, the following table sets forth
as of March 31,  2000  information  concerning  the  Company's  other  executive
officers:

           Name                   Age             Position with BioMarin
           ----                   ---             ----------------------
Raymond W. Anderson                58   Chief Financial Officer, and Vice
                                         President, Finance and Administration
Christopher M. Starr, Ph.D.        47   Vice President, Research and Development
John L. Jost, Ph.D.                55   Vice President, Manufacturing
Emil D. Kakkis, M.D., Ph.D.        39   Vice President of BioMarin and
                                         President, BioMarin Genetics, a
                                         division of BioMarin
Stuart J. Swiedler, M.D., Ph.D.    44   Vice President, Scientific and
                                         Clinical Affairs
Brian K. Brandley, Ph.D.           43   Vice President of BioMarin and
                                          Managing Director, Glyko, Inc., a
                                          wholly-owned subsidiary of BioMarin

Raymond W. Anderson has served as Chief  Financial  Officer and Vice  President,
Finance and  Administration  since June 1998.  Mr.  Anderson  served as the Vice
President,  Finance and Chief Financial Officer at Fusion Medical  Technologies,
Inc., a medical technology  company developing drug delivery systems,  from July
1997 to June 1998. Mr. Anderson served as the Vice President,  Finance and Chief
Financial  Officer  at Fidus  Medical  Technology,  Inc.,  a medical  technology
company specializing in cardiac arrhythmias, from October 1996 to July 1997. Mr.
Anderson served as a director of Recombinant  Capital,  a consulting  firm, from
July 1994 to October 1996. Mr. Anderson  served as the Vice  President,  Finance
and Chief Financial Officer of Glycomed  Incorporated,  a biotechnology company,
from April 1989 to July 1994. Mr.  Anderson was the Chief  Financial  Officer at
Chiron Corporation, a biotechnology company, from 1985 to 1989. Mr. Anderson was
a  Controller  and  Director  of  Financial  Planning  and  Analysis  at  Syntex
Laboratories,  a  pharmaceutical  company,  from 1981 to 1985. Mr.  Anderson has
served as a director of Glyko Biomedical  Ltd. and its predecessor, Glyko, Inc.,
since October 1989. Mr. Anderson  received a B.S. in Engineering from the United
States  Military  Academy,  an M.S. in  Administration  from  George  Washington
University  and  an  M.B.A.   from  the  Harvard  Graduate  School  of  Business
Administration.

Christopher  M. Starr,  Ph.D.  has served as the Vice  President,  Research  and
Development  since the Company's  inception.  From July 1991 to April 1998,  Dr.
Starr has served as the Vice  President,  Research  and  Development  for Glyko,
Inc., a  carbohydrate  analytical  and  diagnostic  company.  Dr. Starr held the
position as National Research Associate at the National  Institutes of Health in
Bethesda,  Maryland from August 1986 to June 1991.  Dr. Starr received a B.S. in
Biology from  Syracuse  University  and a Ph.D.  in  Biochemistry  and Molecular
Biology from the State University of New York, Health Science Center,  Syracuse,
NY.
<PAGE>
John L. Jost, Ph.D. has served as the Vice President,  Manufacturing  since June
1999.  Dr.  Jost  devoted his time from  November  1997 to June 1999 to personal
affairs.   Dr.  Jost  served  in  several   positions  at  Genentech,   Inc.,  a
biotechnology  company,  from February 1983 to November 1997.  From June 1992 to
November 1997, he was Director of Manufacturing Sciences at Genentech. From 1971
to 1983, he served in various scientific positions in process development at The
Upjohn Company, a pharmaceutical company, ending as a Senior Research Scientist.
Dr. Jost received a B.S. and a Ph.D. in Chemical Engineering from the University
of Minnesota.

Emil D.  Kakkis,  M.D.,  Ph.D.  has served as Vice  President  of  BioMarin  and
President of BioMarin  Genetics,  a division of BioMarin,  since September 1998.
From July 1994 to August  1998,  Dr.  Kakkis was a Physician  Specialist  at the
Department of Pediatrics at Harbor-UCLA  Medical Center.  From July 1991 to June
1994,  Dr.  Kakkis  completed a Fellowship  in Genetics at the UCLA  Intercampus
Medical Genetics  Training  Program.  Dr. Kakkis received a B.A. in Biology from
Pomona  College and received a Ph.D.  in  Biological  Chemistry  from UCLA.  Dr.
Kakkis received an M.D. from UCLA.

Stuart J. Swiedler,  M.D.,  Ph.D. has served as Vice President of Scientific and
Clinical  Affairs since June 1998. From November 1997 to June 1998, Dr. Swiedler
was as an independent biotechnology  consultant.  From February 1993 to November
1997,  Dr.  Swiedler  has  served,   in  chronological   order,   with  Glycomed
Incorporated, a biotechnology company, as Assistant Vice President, Biology from
February 1993 to July 1994, as Assistant Vice President, Research from July 1994
to May 1995, and as Vice President, Research from May 1995 to November 1997. Dr.
Swiedler  received a B.S. in Biology  from the State  University  of New York at
Albany. Dr. Swiedler received an M.D. and a Ph.D. in Biochemistry from the Johns
Hopkins University School of Medicine.

Brian K. Brandley,  Ph.D. has served as Vice President of BioMarin since October
1998.  Dr.  Brandley  has served as the  Managing  Director  of Glyko,  Inc.,  a
carbohydrate  analytical  and  diagnostic  company,  since April 1998. He was an
Assistant Professor at Rush University from July 1995 to April 1998, and was the
Senior Scientist, Head of Cell Biology at Glycomed Incorporated, a biotechnology
company,  from July 1988 to July 1995. Dr. Brandley  received a B.S. and an M.S.
in Biology  from the  University  of Miami.  Dr.  Brandley  received a Ph.D.  in
Biology from the University of Sydney, Australia.
<PAGE>


EXECUTIVE COMPENSATION

Summary  Compensation  Table.  The following  table sets forth all  compensation
awarded  to,  earned by, or paid for  services  rendered  to the  Company in all
capacities  during the year ended December 31, 1999, by (1) the Company's  chief
executive  officer  and (2) the other  four most  highly  compensated  executive
officers  other  than the chief  executive  officer  in the  fiscal  year  ended
December 31, 1999,  collectively,  the "Named Executive  Officers".  The entries
under the column "All Other  Compensation"  in the table  represent the premiums
paid for life  insurance  benefits for each Named  Executive  Officer and in the
case of Mr. Denison, also include costs for temporary housing.
<TABLE>


<CAPTION>
                                          Fiscal 1999 Summary Compensation Table

                                                                                  Long-Term        Short-Term
                                                                                Compensation      Compensation
                                                                                  Number of         Number of
                                                                              Shares Underlying Shares Underlying
                                                                                  Options      Options Granted(#)
                                        1999 Annual Compensation  All Other     Granted(#)(1)          (1)
Name and Principal Position            Salary($)     Bonus($)   Compensation($)

<S>                                    <C>           <C>        <C>             <C>              <C>
Grant W. Denison, Jr..................     257,143     257,143       33,045          150,000              4,574
   Chief Executive Officer

John C. Klock, M.D....................     250,000     250,000        2,142          112,500              8,746
   President

Christopher M. Starr, Ph.D............     150,000     150,000          876           75,000             19,189
   Vice President, Research and
   Development

Emil D. Kakkis, M.D., Ph.D............     225,000          --          336           54,167             10,335
   Vice President of BioMarin,
   President of BioMarin Genetics, a
   division of the Company
Raymond W. Anderson...................     189,625     100,000        2,352           62,500             10,158
   Chief Financial Officer and
    Vice President, Finance and
    Administration
<FN>

(1) Long-term options vest 6/48ths at June 30, 1999 for options granted in
     January 1999 and 1/48th per month thereafter and 6/48ths at June 30, 2000
     for options granted in December 1999 and 1/48th per month thereafter.

    Short-term options vest 50% on January 1, 1999, 25% on March 31, 1999 and
     25% on June 30, 1999 for options granted in January 1999 and 25% per
     calendar quarter for options granted in December 1999.  Short-term options
     for Mr. Denison, Dr. Klock and Dr. Starr vest 100% on December 31, 1999.

    Includes the following options granted in January 1999 for services
     rendered to the Company in 1998:

     To Mr. Denison - Long-term Compensation Options to purchase 100,000 shares;
     To Dr. Klock -   Long-term Compensation Options to purchase  75,000  shares;
     To Dr. Starr - Long-term Compensation Options to purchase 50,000  shares;
     To Dr. Kakkis -Long-term Compensation Options to purchase 16,667 shares and
      Short-term Compensation Options to purchase 3,708 shares;
     To Mr. Anderson - Long-term Compensation Options to purchase 25,000 shares
      and Short-term Compensation Options to purchase 4,573 shares.
</FN>
</TABLE>
<PAGE>
Stock Option Grants Table.  The following  table sets forth certain  information
for each grant of options to purchase the  Company's  common stock during fiscal
1999 to each of the Named  Executive  Officers.  All these  options were granted
under  the  1997  Stock  Plan  and have a term of five  years  subject  to early
termination in the event the officer's services to the Company cease.
<TABLE>

<CAPTION>
                                                                    Fiscal 1999 Stock Option Grants



                               Number of     Percent of
                               Securities   Total Options                                   Potential Realizable Value at
                               Underlying    Granted to      Exercise                       Assumed Annual Rates of Stock
                                Options       Employees     Price Per      Expiration       Price Appreciation for Option
                                Granted     in 1999 (1)    Share($) (2)       Date                    Term (3)
                                --------    -------------  ------------     -------     --------------------------------------
                                                                                                   5%                10%
                                                                                                   --                ---
           Name
<S>                             <C>         <C>            <C>              <C>         <C>                    <C>

Grant W. Denison, Jr.               100,000             5%        $ 7.00     12/31/03        $  728,220         $1,020,318
                                     50,000                       $12.75     12/31/04        $  112,315         $  308,675
                                      4,574                       $11.75     12/31/04        $   14,849         $   32,812
John C. Klock, M.D.                  75,000             4%        $ 7.00     12/31/03        $  546,165         $  765,238
                                     37,500                       $12.75     12/31/04        $   84,237         $  231,506
                                      8,746                       $11.75     12/31/04        $   28,392         $   62,739
Christopher M. Starr, Ph.D.          50,000             3%        $ 7.00     12/31/03        $  364,110         $  510,159
                                     25,000                       $12.75     12/31/04        $   56,158         $  154,337
                                     19,189                       $11.75     12/31/04        $   62,293         $  137,652
Emil D. Kakkis, M.D., Ph.D.          20,375             2%        $ 7.00     12/31/03        $  148,375         $  207,890
                                     44,127                       $12.75     12/31/04        $   99,123         $  272,418
Raymond W. Anderson                  29,573                       $ 7.00     12/31/03        $  215,356         $  301,738
                                     43,085             3%        $12.75     12/31/04        $   96,782         $  265,985
<FN>

     (1) Based on an aggregate of 2,877,430 shares subject to options granted by
          the Company during 1999 to employees, consultants and the Named
          Executive Officers.

     (2)  Options  were  granted at an  exercise  price equal to the fair market
          value of the common stock,  as determined by the Board of Directors,
          on the date of grant or by the closing price on the Nasdaq National
          Market.

     (3) The  5%  and  10%  assumed  annual  rates  of  compounded  stock  price
         appreciation  are  mandated  by rules of the  Securities  and  Exchange
         Commission.  The Company  cannot  assure any  executive  officer or any
         other holder of its securities that the actual stock price appreciation
         over the option term will be at the assumed 5% and 10% levels or at any
         other  defined  level.  Unless  the market  price of its  common  stock
         appreciates  over the option term,  no value will be realized  from the
         option grants made to the executive officers.  The potential realizable
         value is  calculated by assuming that the closing price on December 31,
         1999 of $11.75  per share  appreciates  at the  indicated  rate for the
         entire  term of the  option  and that the  option is  exercised  at the
         exercise price and sold on the last day of its term at the  appreciated
         price.  The  potential  realizable  value  computation  is  net  of the
         applicable  exercise price,  but does not take into account  applicable
         federal or state income tax  consequences  and other expenses of option
         exercises  or sales  of  appreciated  stock.  The  values  shown do not
         consider   non-transferability  or  termination  of  the  options  upon
         termination of such employee's employment with the Company.
</FN>
</TABLE>
<PAGE>
Fiscal Year-End Option Value Table. The following table sets forth the number of
shares covered by both exercisable and unexercisable  stock options held by each
of the Named  Executive  Officers at December 31, 1999.  No shares were acquired
upon the exercise of stock options during 1999.
<TABLE>

<CAPTION>
                                                Fiscal Year-End Option Value Table

                                             Number of Securities              Value of Unexercised
                                            Underlying Unexercised             In-the-Money Options
                                              Options at Year-End                at Year-End (2)

                                       Exercisable      Unexercisable     Exercisable     Unexercisable
                Name

<S>                                   <C>               <C>               <C>             <C>
Grant W. Denison, Jr.                         279,574            225,000     $2,056,250         $1,518,750
John C. Klock, M.D.                           214,996            168,750     $1,542,188         $1,139,063
Christopher M. Starr, Ph.D.                   156,689            112,500     $1,028,125          $ 759,375
Emil D. Kakkis, M.D., Ph.D.                    82,875            137,500      $ 618,655         $1,028,126
Raymond W. Anderson                           127,490            102,083      $ 955,576          $ 734,896
<FN>

(1)      Based on closing price on December 31, 1999 of $11.75 per share less exercise price per
            share.
</FN>
</TABLE>

Employment Agreements

We are party to employment  agreements with each executive  officer on the terms
enumerated on the chart below. Each of these employment agreements is terminable
without  cause by the  Company  upon six  months  prior  written  notice  to the
officer,  or by the  officer  upon  three  months  prior  written  notice to the
Company. The Company is obligated to pay the officer's salary and benefits until
this  termination.  In the event that the  officer is  involuntarily  terminated
within one year of a change of control of the  Company,  he will  receive  (i) a
severance  payment equal to six months of his annual salary;  (ii) a bonus equal
to 50% of the annual  bonus that he would  otherwise  be entitled  to, and (iii)
immediate  vesting of 50% of the unvested portion of his outstanding  options to
purchase Company capital stock.


<PAGE>

<TABLE>

<CAPTION>

                                        1999                            Initial Grant of Right          Agreement
                                       Annual                                 to Purchase              Termination
Name of Executive Officer            Salary Rate     Annual Bonus          Equity Securities              Date
- -------------------------            -----------     ------------          -----------------              ----
<S>                                  <C>          <C>                <C>                             <C>
Grant W. Denison, Jr.                 $257,143    Based upon the     1,300,000 shares of             June 30, 2000
                                                  Company's          the Company's common stock      (as amended)
                                                  market             at a purchase price of $1.00
                                                  capitalization.    per share.

John C. Klock, M.D.                   $250,000    Based upon the     800,000 shares of               June 30, 2000
                                                  Company's          the Company's common stock      (as amended)
                                                  market             at a purchase price of $1.00
                                                  capitalization.    per share.

Christopher M. Starr, Ph.D.           $150,000    Based upon the     400,000 shares of               June 30, 2000
                                                  Company's          the Company's common stock      (as amended)
                                                  market             at a purchase price of $1.00
                                                  capitalization.    per share.

Brian K. Brandley, Ph.D.              $139,500    Annual bonus,      Option to purchase up to             None
                                                  payable in         150,000 shares of
                                                  cash or stock,     Glyko Biomedical Ltd.'s
                                                  customarily        common stock.  Now
                                                  between 10-15% of  exercisable for 65,415
                                                  his annual salary. shares of our common stock.

Raymond W. Anderson                   $189,625    Eligible to        Option to purchase                   None
                                                  receive a cash     200,000 shares of
                                                  bonus.             common stock.

John L. Jost, Ph.D.                   $200,000    Eligible to        Option to purchase                   None
                                                  receive a cash     200,000 shares of
                                                  bonus.             common stock.

Emil Kakkis, M.D., Ph.D.              $225,000    Contingent upon    Option to purchase                   None
                                                  regulatory filings 200,000 shares of
                                                  and approvals.     common stock.

<PAGE>
                                        1999                            Initial Grant of Right          Agreement
                                       Annual                                 to Purchase              Termination
Name of Executive Officer            Salary Rate     Annual Bonus          Equity Securities              Date
- -------------------------            -----------     ------------          -----------------              ----

Stuart Swiedler, M.D., Ph.D.           $154,000   Annual bonus,      Option to purchase                   None
                                                  payable in         150,000 shares of
                                                  cash or stock,     common stock.
                                                  customarily
                                                  between 10-15% of
                                                  his
                                                  annual salary.
</TABLE>

We provided a three-year  loan to each of Mr.  Denison,  Dr. Klock and Dr. Starr
for the purchase of their shares  referenced  in column four of the table above.
Each loan bears interest at a rate of 6%. For each of them, respectively, if his
employment is  terminated by us for any reason,  he has the right to sell any or
all of these  shares  of common  stock to us at a price  per share  equal to the
lesser of the  then-current per share market price of the shares or the original
per share purchase price,  $1.00. In the event he ceases  employment with us for
any reason,  we also have the right,  but not the obligation,  to repurchase the
unvested  portion  of the shares at their  original  per share  purchase  price,
$1.00.  Fifty  percent  of the  shares  vested  one year  after  the date of his
employment, with the remainder vesting at a rate of 1/24 per month thereafter.

Dr.  Brandley's  employment  agreement was originally  with Glyko,  Inc. but was
assigned to us in connection with our acquisition of Glyko, Inc.

The founder's annual cash bonus for each of Mr. Denison, Dr. Klock and Dr. Starr
is based on the  difference  between a  minimum  market  capitalization  and the
Company's quarterly market  capitalization.  The annual cash bonus is calculated
as follows:

The Board of Directors  established  a minimum  market  capitalization  of $20.0
million  for the  first  quarter  of 1998.  The  minimum  market  capitalization
increases  by $1.0  million per quarter  until the end of the  agreement  in the
second quarter of 2000. Our quarterly market capitalization is calculated at the
end of each  calendar  quarter by  multiplying  the number of our common  shares
outstanding times the average closing price of our common stock for the last ten
trading  days of the quarter.  If our common  stock is not  publicly  traded the
quarterly market  capitalization  is determined by multiplying the shares of our
common stock  outstanding  by the price at which of our common stock was sold in
the latest significant  investment by an independent  third-party investor.  For
each full $5.0  million that the  quarterly  market  capitalization  exceeds the
minimum market capitalization,  the founders each receive a cash bonus of $1,200
in the first calendar quarter, $1,250 in the second and third calendar quarters,
and $1,300 in the fourth calendar  quarter.  Each founder's annual cash bonus is
the sum of all the four quarterly bonuses.

Each founder's total annual cash bonus may not exceed 100% of base salary in any
year.  Additional  amounts  beyond the cash limit that may be earned in the year
will be paid in stock options using the  Black-Scholes  option  pricing model to
calculate the value of the stock option based on year-end parameters.
<PAGE>
There are no adjustments in the founders' annual base  salaries that result from
increases in market capitalization.

In December 1998, the Board approved a form of  indemnification  agreement to be
entered  into  between  us  and  each  of  our  officers  and  directors.   This
indemnification agreement requires us, among other things, to indemnify officers
and directors  against  liabilities that may arise by reasons of their status or
performance  of their  duties as  officers  or  directors  and to advance  their
expenses  incurred as a result of any  proceeding  against them as to which they
could be indemnified.

For a description  of other  transactions  between the Company and affiliates of
the  Company,  see  "--Certain  Relationships  and  Related  Transactions;   and
- --Compensation Committee Interlocks and Insider Participation."
Section 162(m)

The Company has considered the potential future effects of Section 162(m) of the
Internal  Revenue  Code  on the  compensation  paid to the  Company's  executive
officers,  Section  162(m)  disallows  a tax  deduction  for any  publicly  held
corporation  for individual  compensation  exceeding $1.0 million in any taxable
year  for  any  of  the  Names  Executive   Officers,   unless  compensation  is
performance-based.  The  Company  has adopted a policy  that,  where  reasonably
practicable,  the Company will seek to qualify the variable compensation paid to
its executive  officers for an exemption from the  deductibility  limitations of
Section 162(m).

Employee Benefit Plans

1997 Stock Plan. On November 14, 1997, the Board adopted the 1997 Stock Plan and
approved  the  reservation  of a total of  3,000,000  shares of common stock for
issuance  under the 1997 Stock Plan.  The  stockholders  approved the 1997 Stock
Plan in April 1998.  In December  1998,  the Board  approved an amendment to the
1997 Stock Plan. The stockholders approved the amendment as of January 15, 1999.
The  amendment  increases the number of shares  reserved for issuance  under the
1997  Stock Plan to an  aggregate  of  5,000,000.  The  amendment  also added an
"evergreen  provision"  providing for an annual increase in the number of shares
that  may be  optioned  or sold  under  the 1997  Stock  Plan  without  need for
additional Board or stockholder actions,  which increase shall be the lesser of:
(1) 4% of the  then-outstanding  capital  stock of the  Company,  (2)  2,000,000
shares, or (3) a lower amount set by the Board. The 1997 Stock Plan provides for
the grant of stock  options  and the  issuance  of stock by the  Company  to its
employees,  officers, directors and consultants. The 1997 Stock Plan permits the
grant of options that are either incentive stock options, or ISOs, as defined in
Section 422 of the Internal  Revenue Code of 1986, as amended,  or  nonqualified
stock options, or NSOs, on terms, including the exercise price, which may not be
less than 85% of the fair  market  value of our common  stock for NSOs,  and the
vesting  schedule,  determined  by  the  Board,  subject  to  certain  statutory
limitations and other limitations in the 1997 Stock Plan.

Options granted under the 1997 Stock Plan are generally not  transferable by the
optionee.  Options granted under the 1997 Stock Plan must generally be exercised
within  three  months  after the end of the  optionee's  status as an  employee,
director  or  consultant  of the  Company,  or within  twelve  months  after the
optionee's  termination by death or  disability,  but in no event later than the
expiration of the option's term.
<PAGE>
The exercise price of all incentive  stock options  granted under the 1997 Stock
Plan must be at least equal to the fair  market  value of the  Company's  common
stock on the date of grant.  The exercise  price of NSOs granted  under the 1997
Stock Plan is determined by the Board at an exercise  price not less than 85% of
fair market  value.  With  respect to  nonstatutory  stock  options  intended to
qualify as "performance-based compensation" within the meaning of Section 162(m)
of the Internal  Revenue Code,  the exercise price must be at least equal to the
fair  market  value of the  Company's  common  stock on the date of grant.  With
respect to any  participant  who owns stock  possessing  more than 10% of voting
power of all classes of the Company's  outstanding  capital stock,  the exercise
price of any  incentive  stock option  granted must be at least equal to 110% of
the fair  market  value on the grant  date and the term of the  incentive  stock
option must not exceed five years.  The term of all other options  granted under
the 1997 Stock Plan may not exceed ten years.

The 1997 Stock Plan  provides  that in the event of a merger of the Company with
or into another  corporation,  or a sale of  substantially  all of the Company's
assets,  each option may be assumed or an equivalent  option  substituted for by
the  successor  corporation.  If the  outstanding  options  are not  assumed  or
substituted,  the administrator shall provide for the optionee to have the right
to exercise the option as to all of the optioned stock,  including  shares as to
which it would not otherwise be exercisable.

1998 Employee Stock  Purchase Plan. In December 1998, the Board adopted,  and as
of January 15, 1999, the stockholders approved, the 1998 Employee Stock Purchase
Plan. A total of 250,000  shares of Company  common stock has been  reserved for
issuance  under the 1998 Employee Stock Purchase Plan. The plan also contains an
"evergreen  provision"  providing for an annual increase in the number of shares
which  may be sold  under  the  plan  equal  to the  lesser  of (1)  0.5% of the
then-outstanding  Company  capital stock,  (2) 200,000  shares,  or (3) a lesser
amount set by the Board.

As of December 31, 1999 no shares have been issued under the 1998 Employee Stock
Purchase Plan. However, employee salary withholding totaled $92,464 at March 31,
2000 and will be applied to the purchase of stock under the 1998 Employee  Stock
Purchase Plan as of April 30, 2000.

The 1998 Employee  Stock  Purchase Plan is intended to qualify under Section 423
of the Internal  Revenue Code and contains  consecutive,  overlapping,  24 month
offering periods. Each offering period includes four six-month purchase periods.
The offering periods  generally start on the first trading day on or after May 1
and November 1 of each year, except for the first offering period that commences
on the first trading day or July 23, 1999 and ends on the last trading day on or
before April 30, 2000.

Employees are eligible to  participate if they are  customarily  employed by the
Company or a  participating  subsidiary  for at least 20 hours per week and more
than five months in any calendar  year.  However,  any employee who either:  (1)
<PAGE>
immediately  after grant owns stock  possessing 5% or more of the total combined
voting  power or value of all classes of the  Company's  capital  stock,  or (2)
whose  rights to  purchase  stock  under  all of the  Company's  employee  stock
purchase  plans accrue at a rate which  exceeds  $25,000 worth of stock for each
calendar  year,  may not be granted an option to  purchase  stock under the 1998
Employee  Stock  Purchase  Plan.  The 1998 Employee  Stock Purchase Plan permits
employees to purchase common stock through payroll deduction of up to 10% of the
employee's  compensation,  defined as base earnings and  commissions,  excluding
overtime,  shift premium,  incentive payments and bonuses. The maximum number of
shares an employee may purchase during a single purchase period is 5,000 shares.

The price of stock  purchased  under the 1998  Employee  Stock  Purchase Plan is
generally 85% of the lower of the fair market value of the common stock:  (1) at
the beginning of the offering  period or (2) at the end of the purchase  period.
In the event the fair market value at the end of a purchase  period is less than
the fair market value at the  beginning of the offering  period,  the  employees
will be  withdrawn  from the current  offering  period  following  exercise  and
automatically re-enrolled in a new offering period. The new offering period will
use the lower fair market value as of the first date of the new offering  period
to determine the purchase price for future purchase  periods.  Employees may end
their participation at any time during an offering period, and they will be paid
their  payroll  deductions  to  date.   Participation  ends  automatically  upon
termination of employment with the Company.

Rights  granted  under the 1998 Employee  Stock  Purchase Plan are generally not
transferable  by an  employee  other  than by will or the  laws of  descent  and
distribution.  In the  event of a merger  of the  Company  with or into  another
corporation  or a  sale  of  substantially  all of the  Company's  assets,  each
outstanding option under the 1998 Employee Stock Purchase Plan may be assumed or
substituted  for by the  successor  corporation.  If the  successor  corporation
refuses to assume or substitute for the outstanding options, the offering period
then in progress will be shortened and a new exercise date will be set.

The Board of Directors has the authority to terminate or amend the 1998 Employee
Stock  Purchase  Plan to the extent  necessary  to avoid  unfavorable  financial
accounting  consequences by altering the purchase price for any offering period,
shortening  any  offering  period  or  allocating  remaining  shares  among  the
participants. The 1998 Employee Stock Purchase plan will terminate automatically
ten years  from the  effective  date of this  offering  unless it is  terminated
sooner by the Board.

401(k)  Plan.  The Company  sponsors  the Glyko  Retirement  Savings Plan or the
401(k) Plan. As of January 1, 2000, the plan was renamed the BioMarin Retirement
Savings Plan. Employees are eligible to participate following the start of their
employment,  on the earlier of the next occurring  January 1, April 1, July 1 or
October 1.  Participants may contribute up to approximately 15% of their current
compensation,  up to a statutorily  prescribed annual limit, to the 401(k) Plan.
Each participant is fully vested in his or her salary  reduction  contributions.
Participant  contributions  are  held in trust as  required  by law.  Individual
participants  may direct the  trustee to invest  their  accounts  in  authorized
investment alternatives. The Company pays the direct expenses of the 401(k) Plan
but does not currently match or make  contributions  to employee  accounts.  The
401(k) Plan is intended to qualify under Section 401(a) of the Internal  Revenue
Code  so that  contributions  to the  401(k)  Plan,  and  income  earned  on the
contributions,  are not taxable to  participants  until withdrawn or distributed
from the 401(k) Plan.

<PAGE>



Director Compensation

Directors do not receive cash  compensation  for their  services as directors of
the Company  but are  reimbursed  for their  reasonable  expenses  in  attending
meetings of the Board and while  performing  services for the Company.  Prior to
the effectiveness of the 1998 Director Option Plan, the Company had granted each
non-employee  director an option to  purchase  20,000  shares of Company  common
stock with an exercise price set at the fair market value on the dates of grant,
which was $1.00,  upon their  election to the Board as  consideration  for their
willingness to sit on the Board.  In March 1999,  under the 1998 Director Option
Plan, the Company also issued to each non-employee director an additional option
to purchase 15,000 shares of common stock with an exercise price set at the fair
market value on the date of grant,  which was $7.00, as consideration  for their
ongoing services to the Company as directors. In March 1999 under the 1997 Stock
Plan,  Mr. Sager and Dr.  Gadicke were each also issued an option to purchase an
additional 20,000 shares of common stock of the Company at an exercise price set
at the fair market value on the date of grant, which was $7.00, as consideration
for  services  rendered  by them to the Company in  connection  with the initial
public  offering  completed  in July  1999.  In  November  1999,  under the 1998
Director  Option Plan,  the Company  also issued to Mr.  Sager and Mr.  Williams
subsequent  options to purchase  15,000  shares of common stock with an exercise
price set at the fair market value  (closing price of common stock on the Nasdaq
National Market) on the date of grant,  which was $13.375 as  consideration  for
their ongoing services to the Company as directors.  In December 1999, under the
1998 Director  Option Plan,  the Company also issued to Dr. Gadicke a subsequent
option to purchase  15,000 shares of common stock with an exercise  price set at
the fair market  value  (closing  price of common  stock on the Nasdaq  National
Market) on the date of grant, which was $11.75, as consideration for his ongoing
services to the Company as a director.

1998  Director  Option Plan

The 1998 Director  Option Plan was adopted by the Board in December 1998. It was
approved by the  stockholders  as of January 15, 1999. The plan provides for the
grant of  nonstatutory  stock  options  to  non-employee  directors.  A total of
200,000  shares of Company common stock has been reserved for issuance under the
plan.  The plan also  provides  for an annual  increase in this number of shares
equal to the lesser of: (1) 0.5% of the Company's outstanding capital stock, (2)
200,000 shares, or (3) a lesser amount determined by the Board.

In 1999, options to purchase 90,000 shares were issued to directors.

The 1998 Director  Option Plan provides that each  non-employee  director  shall
automatically  be granted an option to purchase  20,000 shares of Company common
stock on the date which that person first becomes a non-employee director.  This
option shall have a term of ten years. The shares subject to this initial option
shall vest over one year. Each  non-employee  director shall  thereafter also be
automatically  granted an option to  purchase  15,000  shares of Company  common
stock on the anniversary of the date of their respective initial appointments to
<PAGE>
the Board and each anniversary  thereafter,  provided that he or she retains the
Board seat on his or her  anniversary  date.  The shares  subject to this annual
option  shall vest in full one year from the date of grant and shall have a term
of ten years.  These  options  shall  continue  to vest only while the  director
serves.  The exercise  price of each of these  options shall be 100% of the fair
market value of a share of Company common stock at the date of the option.

In the event of a merger or the sale of  substantially  all of the assets of the
Company, each option may be assumed or substituted by the successor corporation.
If an option is assumed or substituted, it shall continue to vest as provided in
the plan.  However,  if a  non-employee  director's  status as a director of the
Company or the successor corporation,  as applicable, is terminated,  other than
upon a voluntary  resignation  by the  non-employee  director,  the option shall
immediately  become fully vested and exercisable.  If the successor  corporation
does not agree to assume or substitute for the option,  each option shall become
fully  vested  and  exercisable  for a period of 30 days from the date the Board
notifies the optionee of the option's  full  exercisability,  after which period
the option shall terminate.

Options granted under the plan must be exercised  within three months of the end
of the optionee's tenure as a director, or within 12 months after termination by
death or  disability,  but in no event later than the expiration of the option's
ten-year term. No option granted under the plan is  transferable by the optionee
other  than by will or the laws of  descent  or  distribution.  Each  option  is
exercisable,  during the lifetime of the optionee, only by the optionee.  Unless
sooner terminated by the Board, the plan will terminate  automatically ten years
from the effective date of the plan.


<PAGE>





PERFORMANCE GRAPH

The following graph compares the Company's  cumulative total stockholder  return
with the  cumulative  total  return of the Nasdaq  Stock  Market  (U.S.) and the
Nasdaq  Pharmaceutical  Index of  stocks in  Standard  Industry  Code  (SIC) 283
encompassing  primarily  biotechnology,  pharmaceutical and medical  specialties
companies,  assuming a $100  investment  in Common  Shares on July 31,  1999 and
reinvestment  of dividends  during the period.  The period  covered by the graph
includes  that  portion of the fiscal year ended  December 31, 1999 during which
the Company was publicly traded.
<TABLE>

<CAPTION>
                        The Company               Nasdaq Stock Market                Nasdaq
                                                         (U.S.)                Pharmaceutical Index

<S>                     <C>                       <C>                          <C>                                           <C>
July 31, 1999                100                          100                          100

August 31, 1999              119                          104                          109

September 30, 1999           112                          100                           94

October 31, 1999              88                          107                          101

November 30, 1999             87                          111                          113

December 31, 1999             88                          122                          128
</TABLE>


The  information  contained  above  under the  captions  "Report of the Board of
Directors on Executive Compensation" and "Performance Graph" shall not be deemed
to be  "soliciting  material" or to be "filed" with the  Securities and Exchange
Commission,  nor shall such  information be  incorporated  by reference into any
future filing under the  Securities  Act of 1933, as amended,  or the Securities
Exchange  Act of 1934,  as  amended,  except  to the  extent  that  the  Company
specifically incorporates it by reference into such filing.


<PAGE>


REPORT ON EXECUTIVE COMPENSATION

The  Compensation  Committee  ("Committee")  is responsible  for setting general
compensation goals and operational guidelines for Company personnel, for setting
all elements of the compensation of the executive  officers of the Company,  and
for approving  grants of stock  options for the Company.  The Committee for 1999
was  composed  of the  three  outside,  non-management  members  of the Board of
Directors.

Compensation Goals and Policies

The goal of the  Company's  compensation  policies  is to  provide  compensation
sufficient to attract,  motivate and retain  executives and staff of outstanding
ability and  potential.  The policies  are intended to establish an  appropriate
relationship  between  executive  compensation  and the creation of  shareholder
value as  measured  by the  equity  markets.  The  Company  uses  the  following
principles to help achieve that goal:

1.       The Company provides competitive compensation packages incorporating
          all compensation elements for executives and staff.

              The Company determines compensation elements in part by the use of
              salary,  stock  option and  benefits  surveys  of  pharmaceutical,
              biotechnology,   and  San  Francisco  Bay  Area  companies.  These
              companies  do not  perfectly  align with  companies  in the Nasdaq
              stock performance graph. The initial  compensation of an executive
              or  senior  staff  member  is set by  negotiation  at the  time of
              recruitment,  but is based on competitive information and internal
              Company  guidelines  and equity.  Based upon surveys,  the Company
              endeavors to provide  compensation  that is equivalent to at least
              the median compensation paid to executives in companies of similar
              size,  location,  type  (pharmaceutical or biotechnology) and with
              comparable levels of experience, achievement,  education/training,
              and responsibility.

2.       The Company rewards executives and senior staff for outstanding
            performance by the individual and by the Company.

              The  Committee  believes  that  a  substantial   portion  of  each
              executive's or senior staff members' compensation should be in the
              form of incentives  for the  achievement of individual and Company
              objectives.  Incentive  compensation  is provided to executives as
              part of the  Company-wide  incentive  plan.  In the period of time
              during which the Company is not profitable,  short-term  incentive
              compensation is preferably paid via stock options valued using the
              Black-Scholes  option  pricing  model.  In  certain  extraordinary
              circumstances, the Board or Committee may authorize the payment of
              cash  bonuses to  recognize  outstanding  performance  on critical
              objectives.  Short-term incentive compensation,  which is reviewed
              and awarded  annually,  is typically  based on both individual and
              Company  performance.  In 1998  (and  until  June 30,  2000),  the
              compensation of the employee-founders,  Mr. Denison, Dr. Klock and
              Dr.  Starr is set by  agreements  made at the time of their  first
              employment which is different than the general practice  discussed
              above. Their compensation  arrangements are discussed under "Chief
              Executive Compensation" below.
<PAGE>
3.       The Company seeks to align the long-term interests of the stockholder
            and the executives and the senior staff.

              The  primary  long-term  compensation  for  all of  the  Company's
              employees is based on the appreciation of the value of their stock
              options. Based on surveys of competitive companies,  all employees
              receive an initial  grant of stock  options upon first joining the
              Company.  The  grant  is  intended  to  be  appropriate  to  their
              organizational  level and is generally in  accordance  with preset
              guidelines or previous levels of grant for similar  positions.  In
              general,  vesting  occurs over four years to maintain a continuing
              incentive  for  executives  and  staff  members  to  remain in the
              service  of  the  Company.  Annually,  the  competitive  level  of
              follow-on  stock options granted after the initial hiring grant is
              determined by surveys for each major position or position level in
              the  Company.  Follow-on  stock  options  intended  for  long-term
              compensation  are  awarded  based  primarily  on these  peer group
              competitive  practices and on Company and individual  performance.
              Promotions also result  typically in an additional  grant of stock
              options to account for the increased  responsibility of the person
              promoted   and  to   reflect   the   option   level  for  the  new
              organizational  level. The Committee believes that these long-term
              compensation practices maintain a continuing incentive to increase
              stockholder  value  as  reflected  in the  equity  markets  and to
              continue   employment  to  vest  and  earn  additional   long-term
              incentives.

Considerations for 1999 Compensation

Increases in base salary for 1999 were made  effective  January  1999  primarily
based  on  the  progress  and  achievements  of the  Company  during  1998.  The
Compensation  Committee took particular note of 1998 achievements  including the
excellent  six month  clinical  trial results of  Aldurazyme(TM)  (then known as
BM101 or  (alpha)-l-iduronidase)  for  MPS-I  reported  in  October,  1998,  the
successful  second round of private  investment  completed in August,  1998, the
acquisition  of  important  related  technology  and an active  business  in the
purchase of Glyko,  Inc. in October,  1998 and the initiation of a joint venture
with Genzyme for  Aldurazyme(TM) in September 1998. The joint venture included a
significant  investment  made by  Genzyme at the  signing  of the joint  venture
agreement.

Based on the Committee's judgment as to the value of these events and other less
visible internal  developments,  the Committee  awarded  long-term  compensation
stock option grants to the executives  and staff of the Company.  Except for the
Company founders,  who were covered under separate  employment  agreements,  the
Committee also granted  short-term  incentive  stock options as a reward for the
Company's  progress in  comparison  to plan in 1998.  Grants under both programs
were  pro rated for the portion of the year that the employee was in the service
of the Company.  Salary compensation across the staff was generally increased by
an average of 5%, which approximated the reported average salary increase in the
San  Francisco Bay Area and was also  pro-rated  for time in service  during the
year. Benefits essentially  remained unchanged.  The Committee deemed that these
Compensation  actions were  appropriate  for the progress made by the Company in
1998 and  maintained  a  competitive  balance  with  biotechnology  companies of
similar size and state of development in the local area.
<PAGE>
Chief Executive Officer Compensation

The  compensation of the three employee founders,  including the Chief Executive
Officer,  Mr. Grant Denison, for 1999 was largely set by bonus formulas in their
employment agreements, as amended, which are effective in this regard until June
30, 2000. The primary  determinant of the founders' annual bonus is based on the
difference  between a minimum  target  market  capitalization  and the Company's
market capitalization as measured quarterly.

Based on the  application of the bonus formula,  the founders  reached the limit
for cash bonus,  which was 100% of base salary.  Additional bonus amounts beyond
the cash limit were paid in stock options using the Black-Scholes option pricing
model  based on the  year-end  market  price and other  market  parameters.  The
maximum  bonus  resulted  from  exceeding  the  targeted   increases  in  market
capitalization by significant  amounts, in part driven by a successful placement
of convertible  notes in April 1999 and by a successful  initial public offering
in July 1999.

In 1999, Mr. Denison earned a maximum cash bonus of $257,143,  which was 100% of
his base  salary.  Mr.  Denison's  base salary was not  increased  in 1999 after
having been adjusted in late 1998 when he went from a partial 70%  commitment to
a full time  commitment to the Company.  After  consideration  of Mr.  Denison's
heavy travel  schedule in 1999, the Committee  authorized the Company to pay the
rent for Mr.  Denison's  temporary  apartment for the eight months in 1999 after
his relocation to the Bay Area. This resulted in other  compensation of $32,805.
The number of Mr.  Denison's  stock options was reduced by options with an equal
value to offset  the  apartment  rent.  Less the  value of the  above  mentioned
apartment  rent, Mr. Denison  received 4,574 stock options as a component of his
1999 performance  bonus.  After  consideration  of the Company's  performance in
1999,  including a successful  private placement and an initial public offering,
Mr.  Denison was granted in December  1999  (priced on the first  trading day of
2000) a long-term equity  compensation  stock option grant for 50,000 shares for
his  contributions  in 1999 and to motivate his  continued  employment  with the
Company.

 From the members of the Compensation Committee of the Company:

                  Erich Sager
                  Ansbert S. Gadicke, M.D., Ph.D.
                  Gwynn R. Williams

<PAGE>

CERTAIN RELATIONSHIPS AND RELATED TRANSACTIONS

Since the Company's  incorporation  in October  1996,  there has not been nor is
there currently  proposed any  transaction or series of similar  transactions to
which the Company was or is to be a party in which the amount  involved  exceeds
$60,000 and in which any director,  executive officer, holder of more than 5% of
the common stock of the Company or any member of the immediate  family of any of
the foregoing  persons had or will have director or indirect  material  interest
other  than (1)  compensation  agreements  and  other  arrangements,  which  are
described  where required in "Employment  Agreements"  and (2) the  transactions
described below.

Transactions with Directors, Executive Officers and 5% Stockholders

On January 15, 1999, the  Compensation  Committee  approved the following option
grants, each at an exercise price of $7.00 per share and vesting 6/48ths on June
30, 1999 and 1/48th per month  thereafter,  to officers of the Company for their
services rendered in 1998:

o  to Mr. Denison, Chief Executive Officer, an option to purchase 100,000 shares

o  to Dr. Klock, President and Secretary, an option to purchase 75,000 shares

o  to Dr. Starr, Vice President, Research, an option to purchase 50,000 shares

o  to Mr. Anderson, Chief Financial Officer and Vice President, Finance and
     Administration, an option to purchase 25,000 shares

o  to Dr. Kakkis, President, BioMarin Genetics (a division of the Company) and
     Vice President of the Company, an option to purchase 16,667 shares

o  to Dr. Swiedler, Vice President, Scientific and Clinical Affairs, an option
      to purchase 23,438 shares

o  to Dr. Brandley, Managing Director of Glyko, Inc. (a wholly-owned subsidiary
      of the Company) and Vice President of the Company, an option to purchase
      12,265 shares

On March 22,  1999,  options  were  granted to Dr.  Gadicke,  Mr.  Sager and Mr.
Williams to purchase 15,000 shares each at an exercise price of $7.00 per share,
vesting  25%  per  quarter   commencing  on  their  first  anniversary  date  of
appointment as a director, as compensation for their services as directors under
the 1998 Director Option Plan.

On March 22, 1999, the Board approved option grants to Dr. Gadicke and Mr. Sager
to  purchase  20,000  shares  each at an  exercise  price of $7.00  per share as
compensation  for services  provided to the management of the Company in regards
to the initial public offering, vesting 100% on July 23, 1999.
<PAGE>
Also on March 22, 1999, the Board approved the following option grants,  each at
an exercise  price of $7.00 per share,  vesting  25% per  calendar  quarter,  to
officers of the Company:

o  to Mr. Anderson, Chief Financial Officer and Vice President, Finance and
       Administration, an option to purchase 4,573 shares

o  to Dr. Kakkis, President, BioMarin Genetics (a division of the Company) and
       Vice President of the Company, an option to purchase 3,708 shares

o  to Dr. Swiedler, Vice President, Scientific and Clinical Affairs,  an option
       to purchase 4,634 shares

o  to Dr. Brandley, Managing Director of Glyko, Inc. (a wholly-owned subsidiary
        of the Company) and Vice President of the Company,  an option to
        purchase 5,005 shares.

On April 12, 1999, the Company sold a total of $26.0 million worth of three-year
promissory  notes  convertible,  according to their terms,  into Company  common
stock,  at an initial  conversion  price,  subject to adjustment,  of $10.00 per
share.  Glyko  Biomedical  Ltd.  purchased $4.3 million worth of these notes and
LaMont Asset  Management  SA    purchased $9.7 million worth of these notes.  In
connection  with this  transaction,  the  Company  also  entered  into an agency
agreement  with  LaMont  Asset  Management  SA,    pursuant to which the Company
agreed to pay LaMont Asset  Management SA a   cash commission of $1.1 million on
sales of notes to  certain  European  purchasers  introduced  to the  Company by
LaMont Asset Management SA.

On June 9, 1999, the Compensation Committee approved the grant to Dr. Jost, Vice
President,  Manufacturing,  of an option to purchase  200,000  shares of Company
common stock at an exercise  price equal to the offering price of $13 per share,
vesting  6/48ths upon the six month  anniversary  of  employment  and 1/48th per
month thereafter.

On July 23, 1999,  concurrent with the Company's IPO, the Company's  convertible
notes payable  (including accrued interest) were converted into 2,672,020 shares
of the Company's  common stock at $10 per share.  Glyko  Biomedical  Ltd.'s $4.3
million  convertible  note plus  interest was  converted  to 441,911  shares and
LaMont Asset  Management  SA's $9.7 million  convertible  note plus interest was
converted to 996,869 shares.

On December 10, 1999, the Board approved the following option grants, each at an
exercise  price of $12.75 per share,  to officers  of the  Company as  long-term
(vests  6/48ths  at June 30,  2000  and  1/48th  per  month  thereafter)  equity
compensation for 1999:

o  to Mr. Denison, Chief Executive Officer,  an option to purchase 50,000 shares

o  to Dr. Klock, President and Secretary,  an option to purchase 37,500 shares

o  to Dr. Starr, Vice President, Research, an option to purchase 25,000 shares
<PAGE>
o  to Mr. Anderson, Chief Financial Officer and Vice President, Finance and
      Administration,  an option to purchase 37,500 shares

o  to Dr. Jost, Vice President, Manufacturing, an option to purchase 18,750
      shares

o  to Dr. Kakkis, President, BioMarin Genetics (a division of the Company) and
      Vice President of the Company, an option to purchase 37,500 shares

o  to Dr. Swiedler, Vice President, Scientific and Clinical Affairs, an option
       to purchase 28,125 shares

o  to Dr. Brandley, Managing Director of Glyko, Inc. (a wholly-owned
       subsidiary of the Company) and Vice President of the Company, an
       option to purchase 28,125 shares

On December 10, 1999, the Board approved the following option grants, each at an
exercise  price of $12.75 per share,  to officers  of the Company as  short-term
(vests 25% per calendar quarter 2000) equity compensation for 1999:

o  to Mr. Anderson, Chief Financial Officer and Vice President, Finance and
      Administration,  an option to purchase 5,585 shares

o  to Dr. Jost, Vice President, Manufacturing,  an option to purchase 2,945
      shares

o  to Dr. Kakkis, President, BioMarin Genetics (a division of the Company) and
      Vice President of the Company, an option to purchase 6,627 shares


o  to Dr. Swiedler, Vice President, Scientific and Clinical Affairs, an option
       to purchase 4,536 shares

o  to Dr. Brandley, Managing Director of Glyko, Inc. (a wholly-owned
       subsidiary of the Company) and Vice President of the Company, an
       option to purchase 4,108 shares

The following  options were granted in lieu of cash bonuses and are fully vested
on December 31, 1999, each at an exercise price of $11.75 per share:

o  to Mr. Denison, Chief Executive Officer,  an option to purchase 4,574 shares

o  to Dr. Klock, President and Secretary, an option to purchase 8,746 shares

o  to Dr. Starr, Vice President, Research, an option to purchase 19,189 shares

On November  30, 1999,  options  were granted to Mr. Sager and Mr.  Williams to
purchase  15,000 shares each at an exercise price of $13.375 per share,  vesting
25% per  quarter  commencing  on  December 1, 1999,  as  compensation  for their
services as directors under the 1998 Director Option Plan.

On December 31, 1999,  an option was granted to Dr.  Gadicke to purchase  15,000
shares at an  exercise  price of $11.75  per  share,  vesting  25% per  calendar
quarter,  as compensation for his services as a director under the 1998 Director
Option Plan.

<PAGE>



                          INDEBTEDNESS OF DIRECTORS AND

                               EXECUTIVE OFFICERS

Other than as described  below, no director or executive  officer of the Company
or associate  of any director or executive  officer is, or at any time since the
beginning of the most recently  completed fiscal year has been,  indebted to the
Company.

Pursuant to the Stock  Purchase  Agreements  under which the Company  sold stock
("Founders'  Shares") to the three founding officers of the Company, the Company
has loaned $1,300,000,  $800,000, and $400,000 to Mr. Denison, Dr. Klock and Dr.
Starr,  respectively,  to purchase  common stock of the  Company.  The loans are
evidenced by an interest  bearing  promissory  notes due on demand and are fully
recourse.

The  following  table sets forth any  indebtedness  of  directors  or  executive
officers  of the  Company  entered  into in  connection  with  the  purchase  of
securities of the Company.
<TABLE>

================================================================================================================================
<CAPTION>
                                                                                                               Security for
                                               Largest Amount of        Outstanding                            Indebtedness
                                                  Outstanding        Indebtedness as of   Number of Common   Number of Shares
                                                Indebtedness (1)   December 31, 1999 (1)  Shares Purchased    of Common Stock
      Name of Borrower            Lender
- --------------------------------------------------------------------------------------------------------------------------------
<S>                               <C>           <C>                <C>                    <C>                <C>
Grant W. Denison, Jr.             Company           $1,475,500             $1,475,500         1,300,000           1,300,000
John C. Klock, M.D.               Company             $908,000               $908,000           800,000             800,000
Christopher M. Starr,
  Ph.D.                           Company             $454,000               $454,000           400,000             400,000
================================================================================================================================
<FN>

(1)      Includes accrued interest at 6% per annum.
</FN>
</TABLE>

SECTION 16(a) BENEFICIAL OWNERSHIP REPORTING COMPLIANCE

Section 16(a) of the Securities  Exchange Act of 1934, as amended (the "Exchange
Act"),  requires the  Company's  directors and officers and persons who own more
that  10% of a  registered  class of the  Company's  equity  securities  to file
reports of ownership and reports of changes in the ownership with the Securities
and Exchange  Commission  (the SEC) and the National  Association  of Securities
Dealers, Inc. Executive officers,  directors,  and greater than 10% stockholders
are required by the SEC to furnish the Company with copies of all Section  16(a)
forms they file.

To be best of the Company's  knowledge,  all reports relating to stock ownership
and such other  reports  required to be file during 1999 under  Section 16(a) by
the Company's  directors,  officers and 10% stockholders were timely filed, with
the exception of Change of Beneficial  Ownership of Securities on Form 4 for Dr.
John C. Klock and Dr. Emil D.  Kakkis in October  1999 and  Schedule  13G for BB
BioVentures L.P. were filed late.
<PAGE>
OTHER MATTERS

Except as otherwise indicated, information contained herein is given as of April
20, 2000. The Company's management knows of no matters to come before the Annual
Meeting  other than the matters  referred to in the Notice of Annual  Meeting of
Stockholders.  However,  if any  other  matters  which  are not now known to the
Company's  management should come properly before the Annual Meeting,  the Proxy
will be voted on such matters in accordance with the best judgment of the person
voting it.

APPROVAL

The contents of this Proxy and the sending thereof to the Stockholders have been
authorized by the Board of Directors of the Company.

DATED at Novato, California this 20th day of April, 2000



/s/ Raymond W. Anderson
Raymond W. Anderson
Chief Financial Officer and

Vice President, Finance and Administration

<PAGE>


                                      PROXY

                          BIOMARIN PHARMACEUTICAL INC.

                       2000 ANNUAL MEETING OF STOCKHOLDERS

                                  JUNE 15, 2000

           THIS PROXY IS SOLICITED ON BEHALF OF THE BOARD OF DIRECTORS

The  undersigned   stockholder  of  BIOMARIN  PHARMACEUTICAL  INC.,  a  Delaware
corporation,  hereby  acknowledges  receipt of the  Notice of Annual  Meeting of
Stockholders and Proxy Statement,  each dated April 20, 2000 and hereby appoints
John  C.  Klock,  M.D.  as  proxy  and  attorney-in-fact,  with  full  power  of
substitution,  on behalf and in the name of the  undersigned,  to represent  the
undersigned   at  the  2000   Annual   Meeting  of   Stockholders   of  BIOMARIN
PHARMACEUTICAL  INC.  to be held on June 15, 2000 at 10 a.m.  local time,  at 46
Galli Drive,  Novato,  California  94949 and at any  adjournment or adjournments
thereof,  and to vote all shares of common stock which the undersigned  would be
entitled to vote if then and there personally  present, on the matters set forth
on the reverse side.

                 (Continued, and to be signed on the other side)



<PAGE>






1.       ELECTION OF DIRECTORS:
         NOMINEES:

           Grant W. Denison, Jr., John C. Klock, M.D.,
           Ansbert S. Gadicke, M.D., Ph.D., Erich Sager
           and Gwynn R. Williams           FOR [   ]     WITHHOLD FOR ALL [   ]

         INSTRUCTION:  To withhold authority to vote for any
         individual nominee, write that nominee's name in the space
         provided below:

         --------------------------------------------

2.       Appointment of Arthur Andersen LLP as independent auditors
         of BIOMARIN PHARMACEUTICAL INC. for the fiscal year
         ending December 31, 2000
                                   FOR [   ]     AGAINST [   ]   ABSTAIN [   ]


3.       In their discretion, the proxies are authorized to vote upon
         such matters as may properly come before the Annual
         Meeting, or any adjournments thereof.
                                   FOR [   ]     AGAINST [   ]   ABSTAIN [   ]



THIS PROXY WILL BE VOTED AS DIRECTED OR, IF NO CONTRARY  DIRECTION IS INDICATED,
WILL BE VOTED FOR THE COMPANY'S NOMINEES FOR ELECTION TO THE BOARD OF DIRECTORS,
THE APPOINTMENT OF ARTHUR ANDERSEN LLP OR AS SAID PROXIES DEEM ADVISABLE ON SUCH
OTHER MATTERS AS MAY PROPERLY COME BEFORE THE ANNUAL MEETING,  INCLUDING,  AMONG
OTHER THINGS, CONSIDERATION OF ANY MOTION MADE FOR ADJOURNMENT OF THE MEETING.

Signature(s)  ___________________________________      Date:  __________________


This proxy should be marked,  dated and signed by the stockholder(s)  exactly as
his or her name appears hereon,  and returned promptly in the enclosed envelope.
Persons signing in a fiduciary  capacity should so indicate.  If shares are held
by joint tenants or as community property, both should sign.


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