Coronary artery ectasia (CAE) is an uncommon form of coronary artery disease. It has been reported in association with a variety of pathological conditions, such as isolated congenital heart disease and Kawasaki disease. CAE is more relevant in young adults with multiple predisposing risk factors, especially familial hypercholesterolemia, and is usually considered a form of atherosclerotic coronary artery disease. A case of CAE is reported with familial hypercholesterolemia and diffuse ectasia of the coronary vessels in association with anomalous origin of the left circumflex coronary artery, which lacked ectatic segments. This combination has not been reported previously.