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Anomalies in the coronary arterial circulation have been described since classical antiquity by Galen and through the Medical Renaissance by Vesalius, but their clinical significance and association with sudden cardiac death (SCD) has only been appreciated over the last 4 decades. Advances in cardiac surgery and cardiovascular intensive care have led to decreasing overall postoperative mortality and morbidity associated with cardiac surgery. The decision whether to surgically treat an anomaly of coronary artery origin and course, and the risk-to-benefit ratio of surgical treatment in preventing a potentially lethal complication of SCD, requires careful, deliberate consideration based on the best available evidence. In this keynote lecture, we aim to deliver a concise discussion of the current surgical management of anomalous coronary arteries.