Unilateral pulmonary vein (PV) atresia is a rare congenital cardiac malformation with evolution toward irreversible pulmonary hypertension. Pneumonectomy or lung transplant is currently the treatment of choice for such a disease. We describe an unusual case of right PV atresia and major aorto-pulmonary collaterals treated with percutaneous angioplasty, stent implantation, and aorto-pulmonary collateral embolization.