Pulmonary lymphangioleiomyomatosis is a disease involving the proliferation of atypical smooth muscle cells trom the perilymphatics, peribronchial and perivascular region of the lung and the retroperitneum. The disease usually affects women of child-bearing age. We recently experienced a case of pulmonary lymphangioleiomyomatosis in a 31-year-old women who had suffered from a chylous pleural effusion. Histologic confirmation of lymphangioleiomyomatosis was made upon a video-associated thoracoscopic lung biopsy. Here we report this case with a brief review of the literature.