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VEXAS syndrome (“vacuoles, E1 Enzyme, X-linked, autoinflammatory, somatic syndrome”) is a recently described autoinflammatory syndrome related to somatic mutations of UBA1. The main clinical features of VEXAS syndrome are heterogeneous, including relapsing fever, relapsing chondritis, and skin lesions such as neutrophilic dermatosis, lung and ocular involvement, venous thrombosis, lymph nodes, and arthralgia. VEXAS syndrome is associated with hematological disease in 50% of cases, mainly myelodysplastic syndrome and monoclonal gammopathy. Treatment depends on whether or not the disease is associated with a hematological disease. When VEXAS syndrome is associated with a myelodysplastic syndrome, hematological treatments, in particular azacitidine, seem to be among the most effective. In the absence of hematological disease, JAK inhibitors appear to be the most effective.