Coronary artery anomalies are not frequent, nevertheless they are associated with increased and potentially lethal cardiac events. Recognition of these anomalies is fundamental in patients undergoing diagnostic or interventional coronary angiography. Most patients presenting with coronary anomalies are asymptomatic, but the risk of myocardial ischemia and sudden death requires the treatment of those patients. Different therapeutic options have been discussed, including surgery, conservative therapy, and interventional approaches. In this report, an aberrant origin of the left main coronary artery arising from the right coronary artery associated with coronary artery atherosclerosis and its surgical correction is described.