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A coronary artery aneurysm is an uncommon vascular disorder, and it can be a life-threatening disease when associated with rupture or an embolism. A 52-year-old man was found to have a 50-mm coronary artery aneurysm at the right coronary artery, and the aneurysm was completely occluded by a thrombus. He had no symptoms after arriving at our hospital, and his hemodynamics was stable. Therefore, initially, we administered anticoagulation therapy involving heparin. After therapy, the distal coronary artery was detected when the thrombus dissolved, and elective surgery was planned. Coronary artery bypass grafting, ligation of the inflow and outflow vessels, and resection of the aneurysm were performed. Early anticoagulation therapy and surgical aneurysm resection were effective for treating the completely occluded coronary artery aneurysm. We herein report this rare case of a giant coronary artery aneurysm occluded completely by a thrombus and treated successfully by anticoagulation therapy and surgical aneurysm resection. [ABSTRACT FROM AUTHOR]