NOTICE TO CONVENE ANNUAL GENERAL MEETING

Copenhagen, Denmark, March 5, 2019 – Orphazyme A/S (ticker: ORPHA.CO), 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:

Wednesday, March 27, 2019 at 5:00 PM (CET)

at the Company’s address Ole Maaløes Vej 3, DK-2200 Copenhagen N, Denmark.

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.