Coronary artery anomalies (CAA) are associated with sudden cardiac death (SCD) and the majority of those events occur during exercise. Depending on the anatomic features and severity, CAA usually provoke clinical symptoms of coronary ischemia, mainly syncope and (exertional) chest pain. Here we present a case of a female adolescent athlete with a high-risk CAA variant and an unusual clinical presentation, which delayed diagnosis 2 years after first symptoms were reported. After successful surgical management of the anomalous artery, the patient was determined eligible for competitive sports with unremarkable follow-up examinations.