Company announcement                                                                                        
Orphazyme A/S
No. 04/2023                                                                                                            
Ole Maaløes Vej 3
DK-2200 Copenhagen N
                                                                                                                                                      
www.orphazyme.com
Company Registration No. 32266355

Copenhagen, Denmark, May 9, 2023 – Orphazyme A/S (ORPHA.CO) (the “Company”), hereby announces the receipt of notification pursuant to Section 38 of the Danish Capital Markets Act from MH Investment ApS, that as of May 8, 2023, MH Investment ApS holds shares, corresponding to more than 5% of the total share capital and voting rights in the Company.

For additional information, please contact

Orphazyme A/S

Anders Vadsholt, Chief Executive Officer and Chief Financial Officer: +45 28 98 90 55