An otherwise healthy 15-year-old girl presented with a history of transient myocardial ischemia. At the time, troponin levels were elevated to 20 microg/L. A myocardial perfusion scintigraphy evaluation revealed a minor scarring with an irreversible inferior defect at rest and at exercise. On admission, the patient was free from symptoms. Transthoracic echocardiography and computer tomography examinations revealed an aberrant origin of the right coronary artery (RCA) from the left sinus of Valsalva, just below the commissure. Repair was accomplished by incision into the RCA and patch plasty. An anomalous aortic origin of a coronary artery from the incorrect coronary sinus of Valsalva is a rare congenital cardiac defect that is associated with an increased risk of sudden death and cardiac morbidity; however, ischemia can occur even when the patient remains asymptomatic. Although this anomalous artery often shares a common orifice with the other coronary artery, it did not in the reported case. In conclusion, because of the high risk of sudden cardiac death, aggressive surgical management is indicated in symptomatic patients.