Single coronary artery is rare congenital anomaly with incidence about 0.04%. Above half of single coronary artery is symptomless, and about 41% of cases are associated with another congenital cardiac anomalies . Single coronary artery is sometimes important for cardiac operation to avoid fatal result from cutting of coronary artery during the right ventriculotomy. Among the associated anomaly of the single coronary artery, coronary arteriovenous fistula is very rare. We experienced congenital single coronary artery with coronary arteriovenous fistula drained into the right ventricle, and so we report this case with literatures.[KTCS 1982;3:366-369]