VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly discovered complicated autoinflammatory syndrome associated with haematological and rheumatological manifestations, recognized for the first time in 2020. In this case report, we describe the first case of VEXAS syndrome in the North Denmark Region. A 76-year-old male was briefly admitted with COVID-19 and a myriad of symptoms including jaw pain, arthralgia, skin rash, malaise, intermittent fever and weight loss. After a prolonged diagnostic evaluation, VEXAS syndrome was suspected and confirmed with the presence of a mutated ubiquitin-like modifier activating enzyme 1 (UBA1) gene.