A 21-year-old man with a history of bronchial asthma during childhood presented with left recurrent pneumothorax. Chest x-ray showed collapse of the left lung. Computed tomography showed hyperlucency and some bullae in the left upper lobe. Thoracoscopic bullectomy and pleurodesis were performed. Pneumothorax recurred twice, for which thoracoscopic bullectomy and pleurodesis were performed. During the third operation, pleurodesis of almost the entire left upper lobe was performed. Since this third operation, the patient has been free from recurrences of pneumothorax for three years. The lung perfusion scintigram performed during the absence of pneumothorax showed a lack of accumulation in the left upper lobe. The lung ventilation scintigram performed during the absence of pneumothorax showed low accumulation and delay of washout in the left upper lobe. These findings are compatible with Swyer-James syndrome. To prevent the recurrence of pneumothorax related to Swyer-James syndrome, some kind of procedure, such as extensive pleurodesis, is necessary.