A newborn female experienced severe respiratory distress immediately after delivery. She presented with intense cyanosis, refractory hypoxemia, and acidosis. The deterioration was rapidly progressive, leading to the child’s demise. The autopsy showed common pulmonary vein atresia, the most severe form of pulmonary venous drainage obstruction. Subpleural and interstitial pulmonary lymphagiectasias also were present as well as evidence of pulmonary hypertension. The lung pathology probably resulted from pulmonary venous atresia during prenatal development.