VEXAS syndrome is adult onset autoinflammatory disease caused by somatic mutations in the gene UBA1 and characterized with overlapping rheumatologic and hematologic clinical features. The most common clinical features included recurrent fever, artralgia/arthritis, pulmonary involvement, skin lessions, vasculitis, and/or thromboembolic event. Aortic-renal aneurysm in VEXAS syndrome is rare entity and its associated with poor prognosis. Herein we described a 69 year-old male patient with VEXAS syndrome with aortic-renal aneurysm responded well to Tocilizumab treatment.