We had expierienced pulmonary lymphangioleiomyomatosis(LAM) with bilateral chylothorax and chylous ascites. A twenty-one-year-old lass with chief complaint of abdominal pain was admitted through the emergency room. She received emergent pelvicoscopic surgery for the rupture of the right corpus luteum. We aspirated 1000ml of the uncoagulated blood. The bleeding point was cautherized electrically. LAM was diagnosed with tissue from the retroperitoneum. Chylous ascites and bilateral chylothorax were occurred despite of various treatments. On thoracotomy, bullous changed lung and lymphatic leakage from visceral and parietal pleura were observed. She died of respiratory insufficiency and general weakness after 6 months from admission. (Korean J Thorac C ardiovasc Surg 2004;37:1029-1031)

Keywords: 1. Lymphangioleiomyomatosis, 2. Chylothorax