We report a rare case of a 6-year-old boy who was diagnosed with coronary artery fistulae communicating with the right ventricle and a left single coronary artery. Preoperative angiography showed a dilated and tortuous single coronary artery draining into the right ventricle. Two coronary artery fistulae draining into the right ventricle were detected at operation and both of these were ligated. Postoperative angiography showed that the single coronary artery diameter was almost normalized, although the vessel was still slightly tortuous. His clinical course was uneventful. In this report, we summarize cases of coronary artery fistula with single coronary artery that have been reported in the literature as well as our case.