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Coronary artery fistulas are rare vascular anomalies characterized by abnormal communication, devoid of a capillary system between the coronary artery and the major vessels or cardiac chambers. In this report, we present a 14-year-old male patient with ischemic symptoms, a left coronary artery to right ventricle fistula and agenesis of the right coronary artery. The anatomy of the coronary arteriovenous fistula was determined in detail through a dual source CT coronary angiography. The patient underwent open cardiac surgery because of ischemic symptoms and a residual fistula was detected after the surgery.