We performed an operation for AAE and AR complicated by ulcerative colitis and aortitis syndrome. The patient was a 20-year-old male who had been treated for ulcerative colitis in our hospital since 1983, when he was 18 years old. In 1985, he was admitted to our hospital for treatment and evaluation of left heart failure. He was diagnosed as having AAE and AR due to aortitis syndrome, and steroid therapy was started. He developed heart failure, and surgery was indicated. At operation, before clamping the aorta we made a composite graft. The ascending aorta and aortic valve were replaced by the composite graft, and button-shaped coronary ostia were sutured directly into the graft. His postoperative course was uneventful and he was discharged. He is now maintained on steroid therapy for his aortitis syndrome.