Anomalous origin of a single coronary artery arising from the innominate artery associated with coronary artery fistula and truncus arteriosus is an extremely rare anomaly. A 16-month-old girl received Rastelli procedure during in the surgical treatment of truncus arteriosus. We found the coronary artery anomaly in the operation. The operation went well, and the patient is doing well without any respiratory symptoms or active intolerance 28 months postoperatively.There may have a connection between a single coronary artery arising from the innominate artery and truncus arteriosus according to the literature reports. The three defects described above are rarely found together, indeed, such a case as ours may be the first reported in the literature.