Five patients underwent operative repair of an aneurysm of the sinus of Valsalva. Four of the aneurysms were considered as congenital and one as mycotic. There were four males and one female. One patient with VSD and characteristics of Marfan's syndrome die on the first postoperative day of a recurrence of fistulae. The follow-up time is over one year for all but one of the surviving patients. There are no late deaths, and all patients are doing well. Aneurysms and fistulae of the sinus of Valsalva, which are perhaps not as rare as was previously thought, present a challenging surgical problem. Ruptured aneurysms and fistulae, even if asymptomatic, should be treated operatively, preferably by a transaortic approach.