Giant coronary artery aneurysms are rare, with a reported prevalence of 0.02% to 0.2%. Causative factors include atherosclerosis, Takayasu arteritis, congenital disorders, Kawasaki disease, and percutaneous coronary intervention. Most giant coronary artery aneurysms are asymptomatic, but some patients present with angina pectoris, sudden death, fistula formation, pericardial tamponade, compression of surrounding structures, or congestive heart failure. Clinical sequelae include thrombus formation, embolization, fistula formation, and rupture. Surgical correction is generally accepted as the preferred treatment for giant coronary artery aneurysms. We present an illustrative case of a giant 70 × 40-mm coronary artery aneurysm in a 56-year-old man who declined surgery and died one month later. In addition, we provide a review of the medical literature on giant coronary artery aneurysms.