Coronary artery fistulas (CAFs) are rare vascular anomalies. We describe a
case of CAF draining into the left atrium, combined with an atrial septum d
efect (ASD) and azygos continuity in a 3-year-old girl. Surgical therapy in
cluded closure of the ASD combined with closure of the CAF through a left a
trial approach. Even though CAF can be treated by interventional means, sur
gical correction may be required in the presence of associated cardiac abno
rmalities.