Anomalous aortic origin of the left coronary artery (AAOLCA) confers high risk for sudden cardiac arrest (SCA). This series aims to describe consecutive admissions with interarterial AAOLCA presenting with SCA and distinct clinical trajectories. An eight-year-old boy collapsed at school and received 10-min of cardiopulmonary resuscitation (CPR) and defibrillation prior to return of spontaneous circulation. He had no end-organ dysfunction and underwent uneventful coronary unroofing. In contrast, a 14-year-old boy presented with collapse while jogging. He received 40-min of CPR prior to extracorporeal membranous oxygenation cannulation with multisystem dysfunction and persistent severely depressed left ventricular function. He is now rehabilitating following uneventful orthotropic heart transplantation. These cases illustrate the diverse outcomes of AAOLCA with SCA following exertional syncope.