A 32-yr-old male patient with recurrent pneumothorax associated with bronchial atresia of the subsegmental branch of the posterior segmental bronchus of the right upper lobe was successfully treated with right upper lobectomy. Before surgery, the bronchial atresia with pneumothorax was suspected on the chest radiograph and CT scans, which showed the findings of bronchocele with localized hyperinflation of the right upper lobe. The examination of surgical specimen from the resected right upper lobe suggests that the cause of the recurrent pneumothorax was the rupture of the subpleural bullae in the hyperinflated lung segment distal to the atretic bronchus.