We describe the case of a 67-year-old man with a rare combination of a giant coronary artery aneurysm with a fistula draining into the coronary sinus. The patient presented with a sensation of retrosternal pressure. He was examined by coronary angiography, which revealed a large aneurysm of the right coronary artery (RCA) with a fistula. This fistula originated from the distal RCA shortly beyond the crux and drained into the coronary sinus. The aneurysm was in the proximal portion of the RCA, measuring 4 cm in diameter and 7 cm in length. Surgical repair by closure of the fistula under direct vision, suture closure and plication of the aneurysm, and coronary artery bypass was performed. Postoperative echocardiography and computed tomography confirmed closure of the fistula. The patient remains symptom-free at 4 months after surgery.