Single coronary artery combined with other cardiac anomalies is uncommon. We present a 64-year-old man with unicuspid valvular and infundibular pulmonary stenosis, single coronary artery arising from right sinus of Valsalva, combined with coronary artery fistula and secundum atrial septum defect. This patient received operation of pulmonary valve replacement, infundibulectomy and ASD closure. He had complete resolution of symptoms after surgery.