Controversy still exists concerning the diagnosis and treatment of congenital lobar emphysema (CLE). Although surgical removal of the affected lobe is the most commonly accepted form of treatment, detection of milder or even asymptomatic cases is usually followed by a more conservative management of patients, i.e. non-surgical treatment and follow-up. We therefore decided to evaluate our patients with CLE, placing special emphasis on treatment and diagnostic techniques. We also evaluated quantitative analyses of alveolar diameters. Fourteen children with CLE were analysed retrospectively, including age, sex, clinical picture, localisation, diagnostic and surgical modalities and histopathologic diagnosis. The alveolar diameters of affected lobes were compared with those of the lobectomised patients with other non-obstructive respiratory diseases. All children but one had severe respiratory distress as an initial symptom. All patients, except newborns, had a history of pulmonary infection. All cases underwent thoracic CT examination as the main radiologic method. In all of the patients, only one lobe was affected. We found an obvious mediastinal shift and atelectasis of adjacent lobes due to compression of the affected lobe. The affected lobe was therefore surgically removed in all of the children. In one case, we had to carry out a partial lobectomy to reduce the duration of the operation, due to an intraoperative fall of oxygen saturation. All of the children had an uneventful clinical course postoperatively. The alveolar diameters of the cases with CLE were significantly greater than those of the control patients. We think that the majority of cases with CLE have too severe respiratory distress to avoid surgical removal of affected lobe. Conservative management should be reserved only for patients with milder symptoms or no distress at all.