Common pulmonary vein atresia is a rare congenital anomaly that is rapidly fatal unless immediately recognized and corrected by surgical intervention. This article describes three neonates who died with the diagnosis soon after birth. In the constellation of presenting clinical features in each case, the occurrence of early spontaneous pneumothorax was particularly noteworthy. This sign may well be a helpful physiologic marker within the context of suspected congenital heart disease associated with severe pulmonary venous obstruction.