Pleural disease is a common complication of lymphangioleiomyomatosis (LAM). The incidence and recurrence rates of secondary spontaneous pneumothorax in LAM are the highest among chronic pulmonary disorders. Most patients have at least one pneumothorax before LAM is diagnosed, and pneumothorax is often the sentinel event that leads to the diagnosis. Although early, definitive treatment for recurrent pneumothorax by pleurodesis is recommended, the failure rate for chemical and surgical approaches is high. Chylothorax occurs owing to obstruction of lymphatics by proliferating smooth muscle cells and often requires pleurodesis to control expanding and recurrent effusions. Because of the rarity of the LAM, few data exist on whether the occurrence of pleural complications in LAM accelerates functional decline or portends a worse prognosis.