Congenital coronary arteriovenous fistula is relatively uncommon and often associated with additional congenital and acquired heart disease. If coronary arteriovenous fistula is suspected, the diagnosis can be made readily by cardiac catheterization and selective coronary arteriography. Surgical treatment is very satisfactory, with a low mortality and apparent good long term result. Recently, we experienced one case of congenital coronary arteriovenous fistula which was associated with aortic stenosis and regurgitation. The tortuous fistula tract was noted between the left anterior descending coronary artery and the main pulmonary artery. Under the cardiopulmonary bypass, aortic valve replacement[Carbomedics 23mm] and suture closure of the draining orifice of coronary arteriovenous fistula in the main pulmonary artery just above the pulmonary valve were performed, Postoperative hospital course was uneventful and the patient was discharged postoperative 9th day without any problems.