NOTICE TO CONVENE ANNUAL GENERAL MEETING

Copenhagen, Denmark, March 19, 2018Orphazyme A/S, a biopharmaceutical company dedicated to developing treatments for patients living with rare diseases, today announced that the Orphazyme A/S Annual General Meeting will be held on:

Thursday, April 12, 2018 at 5:00 PM (CET)

at Gorrissen Federspiel Advokatpartnerselskab, Axeltorv 2, DK-1609 Copenhagen V.

The notice to convene the Annual General Meeting, including Appendix 1: Candidates for the Board of Directors, is attached.

Additional information about the Annual General Meeting is available at Orphazyme A/S’ website, http://phx.corporate-ir.net/phoenix.zhtml?c=254662&p=irol-agm.