Behçet's disease is a systemic vasculitis of unknown etiology, which generally affects young adults and can produce systemic manifestations (ophthalmic, neurological, cardiac, pulmonary, vascular and renal), which are often the final cause of death in these patients.We present the clinical case of a 56-year-old male patient with clinical criteria for the diagnosis of Behçet's disease (oral lesion and recurrent genital ulceration) who presented a ruptured abdominal aortic aneurysm.Surgical treatment was by resection of the aneurysm and repair of the aorta associated with immunosuppressive therapy. The postoperative period evolved satisfactorily.Behçet's disease is frequently associated with vascular manifestations at different levels, so treatment must be individualized.