Isolated coronary artery fistula in the newborn is a very rare congenital anomaly. When symptomatic, treatment options include surgical management with ligation and/or patch closure or transcatheter coil embolization. We describe the case of a newborn with symptomatic left coronary-right ventricular fistula, which was managed operatively with off-pump ligation and on-table angiogram for confirmation of fistula obliteration and normal distribution of flow in the coronary artery. We point out the advantages of on-table angiography to evaluate and potentially treat residual structural pathology after cardiac surgery.