A 50-year-old woman complained of hoarseness and chest X-ray demonstrated a widening of the superior mediastinum. Computed tomography scanning and aortography demonstrated aneurysmal dilatation at the innominate artery. Coronary arteriography showed a coronary pulmonary artery fistula originating from the left anterior descending artery. The surgical procedure was prosthetic bifurcated bypass grafting from the ascending aorta to the right common carotid artery and right subclavian artery. During the period of innominate artery occlusion, the patient was cooled to 25 degrees C with selective cerebroperfusion and circulatory arrest. Pathologic diagnosis of the specimen was that of a true aneurysm with atherosclerotic plaque and calcification. The coronary pulmonary artery fistula was closed inside the pulmonary artery. There were no signs of neurologic deficit noted. Innominate artery aneurysm is uncommon and the surgical approach varies in each case. A coronary artery fistula is also an uncommon congenital malformation but has been recognized more often with the improvement in diagnostic techniques such as selective coronary arteriography. We performed successful surgical treatment for a rare case of innominate artery aneurysm with a coronary pulmonary artery fistula.