Here in nine patients with congenital lobar emphysema who had been treated surgically in the previous 10 years are reported. The ages of the patients at diagnosis ranged from 26 days to 11 months. The six patients whose symptoms started in the neonatal period had more severe dyspnea, cyanosis and respiratory distress. Tube thoracostomy was performed in two of three patients who had been misdiagnosed initially. The affected side was the left upper lobe in five patients, the right upper lobe in three, and the right middle and upper lobes in one patient. Lobectomy was performed in all cases. Dysplasia of the bronchial cartilage was found in six patients and bronchial atresia of the left upper lobe was found in another infant as the etiologic cause of the condition. Although the possibility of conservative management in congenital lobar emphysema has been reported recently, we believe that surgery is the treatment of choice in patients who have persistent or progressive, severe respiratory distress in spite of medical treatment.