A 16-year-old boy with tetralogy of Fallot (TOF), pulmonary stenosis, and coronary artery to pulmonary arterial fistulous communication arising from the proximal right coronary artery is reported for its rarity. The surgical importance of this anomaly is highlighted. The diagnosis should be borne in cases of TOF, with echocardiographic demonstration of severe right ventricular outflow tract obstruction without a corresponding degree of systemic arterial desaturation.
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