VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a rare X-linked autoinflammatory disease that is caused by somatic mutations of the UBA1 gene which leads to a clonal proliferation of myeloid cells.1, 2, 3 The estimated prevalence of VEXAS syndrome is about 1:13,591.2 Here, UBA1 is linked to VEXAS syndrome.