There is a risk for high-output congestive heart failure to be associated with an extra-lobar pulmonary sequestration (ELS) when there is left-to-left shunting caused by a large systemic arterial supply to a sequestration and venous drainage via the pulmonary veins into the left atrium. We present a 2-year-old boy who underwent thoracoscopic resection of an unusually hypervascular right ELS with a high output left-to-left shunt between the aorta and the left atrium via a pulmonary vein. This case is of particular interest because computed tomographic angiography identified a hypervascular nidus with indistinct borders between arterial and venous vessels suggestive of an arteriovenous malformation.