Congenital coronary arteriovenous fistula is relatively uncommon, but with widespread use of cardiac catheterization, angiography and selective coronary arteriography are being recognized with increasing frequency. Recently we experienced one case of congenital coronary artery fistula which originated from the right coronary artery. The case was 25 year-old-male, who complained of dyspnea on exertion and continuous murmur was heard, and diagnosed as right coronary artery fistula by the cardiac catheterization and aortography. On the operation field, the right coronary artery was markedly dilated from aorta to the middle segment at acute margin of the right ventricle, which the hen-egg sized aneurysm was noticed. The dilated coronary ostium and fistular site were obliterated with several horizontal mattress sutures. And the dilated tortuous right coronary artery with aneurysm was excised. Postoperative course was uneventful and discharged without problem.