A 31-year-old woman was transferred to our hospital for treatment of a right pneumothorax. She had presented initially with moderate dyspnea and coughing at a local clinic, where a chest radiograph showed a collapsed right lung. Chest computed tomography showed overinflation of the middle lobe and a large bulla. We diagnosed congenital lobar emphysema of the middle lobe with pneumothorax and performed middle lobectomy by video-assisted thoracic surgery via four ports (5–12 mm in size). The patient had an uneventful postoperative course and was discharged from hospital 5 days after surgery.