A 23-day-old female newborn was evaluated for acyanotic respiratory distress immediately after birth. Echocardiogram and cardiac catheterization revealed absence of the right pulmonary artery. Subsequent course was complicated by necrotizing bronchopneumonia. Despite antibiotic therapy and ventilator support she failed to improve; right pneumonectomy was performed to remove the source of sepsis. This case represents an example in which an infectious complication in a rare congenital pulmonary malformation served as an indication for neonatal pneumonectomy.