Coronary artery anomalies are a diverse group of congenital disorders presenting with highly variable clinical manifestations. The anomalous origin of left circumflex artery from the right coronary sinus following a retro-aortic trajectory is a well-recognized anatomic variation. Despite its benign course, it can prove lethal in association with valvular surgery. When single aortic valve replacement or combined with mitral valve replacement is performed, the aberrant coronary vessel may be compressed by or between the prosthetic rings triggering postoperative lateral myocardial ischemia. If left untreated, the patient is at risk of sudden death or myocardial infarction with its detrimental complications. Skeletonization and mobilization of the aberrant coronary artery is the most widely accepted intervention, but valve downsizing or concomitant surgical or transcatheter revascularization have also been described. However, large series are lacking from the literature. Therefore, no guidelines exist. This study is a thorough review of the literature concerning the aforementioned anomaly in association with valvular surgery.