The vast majority of sinus of Valsalva aneurysm originated from a localized congenital defect of the aortic media and less frequently from infections or degenerative processes affecting the aortic wall. But aneurysm of sinus of Valsalva has not been reported up to the present in a patient with Behçet's disease. We report a 39-year-old woman presenting ruptured aneurysm of the sinus of Valsalva and Behçet's disease.