Congenital coronary arteriovenous fistula is a communication of a coronary artery with one of the atria, ventricles, the coronary sinus, the superior vena cava, or the pulmonary artery. We had a successful surgical experience with 63 year-old-female patient who complained substernal chest pain on exertion for 8 years. On auscultation, a continuous murmur was heard at the left second to third intercostal space along the left sternal border. The right cardiac catheterization was revealed to 4% oxygen step up between right ventricle to main pulmonary artery, and Qp/Qs was 1.3:1. The selective coronary arteriography showed markedly tortuous dilated vessel which originated from left coronary artery draining into the main pulmonary artery. The operation performed to mid portion of tortuous and dilated fistula by multiple ligation with 3-0 Mersilene and suture ligation with pledgetted 3-0 Prolene on distal draining site, Postoperative course were uneventful without any symptoms and complications.