Coronary artery fistula is an uncommon congenital heart defect that is readily amenable to surgical treatment. This fistula usually originates from the right coronary artery, but may arise from the left coronary artery, both coronary arteries, or single coronary artery. And the fistulous communication is most often to right ventricle, right atrium or pulmonary artery. Recently we experienced one case of congenital coronary artery fistula which was associated with patent ductus arteriosus. The fistulous communication, forming aneurysmal dilatation, was noted between the left anterior descending coronary artery and the right ventricular outflow tract. Cardiopulmonary bypass was employed in this case. After an arteriotomy was made on the aneurysmal coronary artery, both the proximal opening and the termination site of the fistulous tract were directly closed with partial aneurysmorrhaphy. The right ventricular chamber was also opened to evaluate the fistulous termination site. Postoperative hospital course of the patient was uneventful and she was discharged without problems.