In this case, we reported a 24-day-old infant with a rare combination of the anomalous origin of coronary artery and the type A interrupted aortic arch, who presented with significant shortness of breath. Besides these anomalies, further cardiac examination including transthoracic echocardiography and CT angiogram revealed ventricular septum defect, patent foramen ovale and patent ductus arteriosus at the same time. Surgical procedures including repair of ventricular septum defect, ligation of patent ductus arteriosus, unroofing of right coronary artery and end-to-side aortic anastomosis were performed to cure the infant. This patient stayed in ICU for few days without any major complications and discharged from hospital at the tenth day after surgery.