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The majority of coronary artery fistulas (CAFs) are congenital. The anomaly accounts for 0.4% of congenital heart defects and approximately 50% of pediatric coronary vasculature anomalies. Twenty percent of people with congenital CAFs have other concomitant cardiac anomalies, most frequently aortic and pulmonary atresia and patent ductus arteriosus. It is worthy of note that CAF with the tetralogy of Fallot has also been reported. Here we describe a patient with a double outlet right ventricle in association with a coronary artery fistula.