Lymphangioleiomyomatosis result from the proliferation of immature smooth muscle cells in the peribronchial, perivascular and perilymphatic areas of the lung. The disease primarily affects women of childbearing age, which has dyspnea, recurrent episodes of pneumothorax, pulmonary edema, chylous effusion and hemoptysis as symptoms. Most patients die from respiratory failure within 10 years after diagnosis. Definite diagnosis depends on histologic findings. We report our experience of anesthetic management for a pregnant woman with lymphangioleiomyomatosis which was confirmed with biopsy of inguinal lymph nodes. (Korean J Anesthesiol 1996; 30: 740-745)