Congenital anomaly of the coronary artery is rare. We have to care about the injury of the aberrant coronary artery and ischemic complication during and after the heart valve surgery. We experienced a good clinical course of aortic valve replacement (AVR) with concomitant coronary artery bypass grafting (CABG) for aortic stenosis coexisting with anomalous aortic origin of the right coronary artery. A 72-year-old woman had suffered from dyspnea and palpitation on effort, and we diagnosed severe aortic stenosis. Preoperative examination revealed the right coronary artery arising from the left coronary sinus with a stenotic lesion in the interarterial course between the aorta and main pulmonary artery. She underwent AVR and CABG using a saphenous vein graft. The peripheral anastomosis of the bypass grafting was performed before starting cardiopulmonary bypass and the blood cardioplegia was infused into right coronary artery through the bypass graft during cardiac arrest. The postoperative course was uneventful and the patent bypass graft was confirmed by computed tomographic angiography.