Cardiac complications, especially aortic regurgitation, are very rare in Behçet's disease. This report describes a patient with a complicated abnormality of the aortic valve apparatus and severe aortic regurgitation secondary to Behçet's disease. In the literature, flail prolapsed valve, perforated valves and ruptured pseudoaneurysms of the sinus of Valsalva have been reported. Echocardiography may be most useful to detect these anatomically complex abnormalities and therefore to elucidate the cause of aortic regurgitation in Behçet's disease.