AbstractHere, we report a four‐day old neonate presenting with cardiovascular collapse secondary to a descending aorta to right atrial fistula. Echocardiography was suggestive of the diagnosis and confirmed with computed tomographic (CT) imaging. The fistula was successfully occluded via cardiac catheterization using an Amplatzer Vascular Plug II implanted from the right atrial aspect. This report includes the first CT imaging of this extremely rare congenital defect as well as detailing the first successful transcatheter occlusion of this particular lesion.