Congenital coronary fistula is a rare condition, and with widespread use of cardiac catheterization, angiography and selective coronary arteriography are being recognized with increasing frequency. Fistula originating from the right coronary artery are more common than those from the left coronary artery. The fistula empties into the right side of the heart in 90% of the cases with the right ventricle being the most common recipient chamber, followed by the right atrium and the pulmonary artery. We report a case of congenital coronary artery fistula of the right coronary artery to the left ventricle with significant shunt in a 20 - year old female. It was detected by transthoracic and transesophageal echocardiography and confirmed by cardiac catheterization and coronary angiography. The fistula opening was closed with 6-0 Prolene continuously under cardiopulmonary bypass and moderate hypothermia [ 28 oC ]. Postoperative course was uneventful and the patient was discharged without specific problem.