Coronary arteriovenous fistula (CAF) is most common form of hemodynamically significant coronary anomaly. CAF combined with atrial septal defect (ASD), however, constitutes an uncommon subgroup of CAF. We present a case of congenital CAF combined with secondum ASD, which are manifested by congestive heart failure. These anomalies were identified by transthoracic echocardiography and coronary angiography respectively. These ASD and CAF were closed by operation successfully without any complication.