A 31-year-old male with tetralogy of Fallot (TF) and total occlusion of the right coronary orifice complicated with infective endocarditis successfully underwent total repair of TF and coronary artery bypass graft (CABG). The patient had severely suffered from symptoms including breathlessness, palpitation (SVT) and chest pain. The coronary arteriography revealed occlusion of the right coronary orifice. The preoperative course was further complicated by endocarditis with vegetation of the aortic valve that did not respond to antibiotics. Concomitant surgical procedures consisting of TF repair, CABG to the right coronary artery with saphenous vein graft and vegetectomy of the aortic valve were carried out. The postoperative course was uneventful though he underwent cholecystectomy for symptomatic gall stones after TF repair. The patient is now in NYHA class II.