The issues surrounding congenital coronary anomalies and their effect on sudden death are complex. Researchers are still trying to fully understand anomalous aortic origin of a coronary artery (AAOCA) and its relationship to adverse health outcomes in humans, especially children. Using the most up-to-date literature, as well as the input of leading experts in the field, the American Association for Thoracic Surgery (AATS) has released practical guidelines for the identification and treatment of AAOCA, including an overview of the latest data surrounding population-based risk.