We report a case of pulmonary lymphangioleiomyomatosis discovered current with a spontaneous pneumothorax. The patient was a 21-year-old female with chest pain. X ray on admission revealed right pneumothorax. Chest CT showed multiple cystic shadows in the whole lung field. Pulmonary lymphangioleiomyomatosis was diagnosed histopathologically by thoracoscopic lung biopsy. Relatively few cysts were observed on the CT, and their sizes were small. Pulmonary function tests were normal. Therefore, this case may be in a relatively early stage. The patiant is currently being treated with progesterone therapy. Unfortunately, though the patient has been treated for about 1 year, cystic shadows on chest CTs have increased in number and size, and pulmonary function data have slightly declined.
