Right coronary artery (RCA) -left ventricular (LV) fistul with a giant right coronary artery aneurysm (CAA) is very rare. We reported a giant right CAA with the widest inner diameter of about 56.6 mm. Its terminal communicated with the LV with an inner diameter of about 22 mm. And the patient had the huge LV. We successfully operated the surgery by suturing the beginning of the coronary aneurysm and performing the coronary artery bypass grafting (CABG). RCA-LV fistul with a giant right CAA had some serious complications such as thrombosis, rupture and heart failure. Therefore, it is necessary to establish a positive and effective treatment strategy. Although our case is not unique, it represents a classic surgical treatment method.