Tetralogy of Fallot associated with ascending aortic aneurysm and aortic valve regurgitation is unusual. This combination necessitates a change in operative strategy during complete tetralogy repair. We present a 43-year-old woman who successfully underwent composite graft replacement of the ascending aorta and aortic valve during primary complete tetralogy repair. Histologic examination of the aorta revealed medial degeneration. (C) 2001 by The Society of Thoracic Surgeons.