The combination of coronary artery aneurysm and pulmonary artery fistula is extremely rare and its common cause is atherosclerosis. A 61-year-old woman presented with a giant coronary artery aneurysm with pulmonary artery fistula and intramyocardial calcifications of the left ventricle associated with progressive atherosclerosis due to chronic hemodialysis. The coronary artery aneurysm was resected under cardiopulmonary bypass because of hemodynamic instability due to restrictive cardiac dysfunction. The patient's restrictive cardiac dysfunction was improved after aneurysm resection. Surgical resection should be considered for giant coronary artery aneurysm with restrictive cardiac dysfunction. (Ann Thorac Surg 2010;89:963-5) (C) 2010 by The Society of Thoracic Surgeons