Survival to seventh decade with tetralogy of Fallot is uncommon. Herein we report a successful repair of tetralogy of Fallot combined with aortic valve replacement for concomitant aortic valve regurgitation in a 65-year-old woman. She had been in severe congestive heart failure and respiratory failure, and also showed liver and renal dysfunction preoperatively. Postoperative course has been good but ventricular arrhythmia of Lown grade II persisted.