We describe the case of a patient in whom evaluation of effort angina revealed a tight stenosis of a right coronary artery anomalously arising from the left sinus of Valsalva, which was successfully treated by stent implantation. The abnormal origin of the right coronary artery from the left aortic sinus coursing between the aorta and the pulmonary trunk is a rare congenital anomaly. It may remain asymptomatic, but can also cause major cardiac events, even in the absence of coronary atherosclerosis. We discuss the clinical importance of this anomaly and review the literature concerning current views and therapy.