Usually lymphangioleiomyomatosis (LAM) progresses gradually and without effective treatment eventually leads to respiratory insufficiency. Lung transplantation remains the only effective treatment for patients with end-stage LAM. Most patients with LAM who are evaluated for lung transplantation have undergone unilateral or bilateral pleural fixation and the International Society for Heart and Lung Transplantation currently considers that a history of pleural fixation is not a contraindication to lung transplantation and that pneumothorax should be managed optimally when it occurs without undue concern for distant lung transplantation.