An extremely rare case of a coronary artery fistula with a concomitant saccular aneurysm is presented. A 65-year-old woman, who had a history of chest bruising 5 years earlier, suffered from chest pain, which was diagnosed as being due to left coronary artery-pulmonary artery fistulae concomitant with a giant saccular coronary artery aneurysm. Suture closure of the afferent coronary artery to the aneurysm, aneurysmorrhaphy, and transpulmonary closure of coronary artery-pulmonary artery fistulae were performed. The postoperative course was uneventful and the patient was well at 3 months after the operation. Because the risk of surgery appears to be less than the potential development of fatal complications, it is recommended for the treatment of coronary artery fistula with a concomitant saccular aneurysm.