10 patients with Marfan's syndrome and cardiovascular disease were operated at Tohoku University Hospital from 1971 to 1988. Surgery included composite valve graft replacement of ascending aortic aneurysm with aortic regurgitation in 5 patients and prosthetic mitral valve replacement in three patients; two had resection of aneurysm with Dacron tube replacement. Operative mortality was 10%. Two late death occurred (22%). It was suggested that regular follow-up examination is important in these patients to detect new lesions and to evaluate known lesion.