Between October, 1979 and September 1994, 18 consecutive patients with Marfan's syndrome underwent replacement of the ascending aorta and aortic valve with a composite graft. There were 11 men and seven women; the mean age was 38.7 years. All patients had annuloaortic ectasia with significant aortic regurgitation. Ten of the 18 patients had aortic dissection. Concomitant procedures were performed in three patients. The hospital mortality rate was 17%, and two patients with concomitant procedures died early after surgery. There were three late deaths, two of which were related to late aortic complications. The actuarial 8-year survival rates in patients with and without aortic dissection were 58.3% and 77.8%, respectively. Since aortic dilatation and its complications are progressive in patients with Marfan's syndrome, improved surgical results can be obtained early in the course of the disease before cardiac disturbances and aortic dissections emerge.