Bronchial atresia is a rare congenital anomaly, and neonatal presentation is extremely rare. Bronchial atresia often affects segmental or subsegmental bronchi, and bronchial atresia of a lobar bronchus is also rare. We report 2 cases of prenatally diagnosed congenital lobar emphysema caused by bronchial atresia at the left upper lobar bronchus. Three-dimensional computed tomography bronchoscopy was effective to demonstrate accurate anatomical changes of the bronchus.