Abstract
Lymphangioleiomyomatosis (LAM) is a rare disease that leads to airways and lymphatic channels obstruction due to abnormal smooth muscle proliferation. It presents with dyspnea, pneumothorax or chylothorax. Lung transplantation (LT) has emerged as a valuable therapeutic option with limited reports. We report a case of LAM that underwent double LT and complicated by refractory bilateral chylothorax which was managed successfully by povidone-iodine pleurodesis and the addition of sirolimus to the post-transplantation immunosuppressive therapy. The patient has no recurrence with 24 months follow-up.