A 39-year-old woman had been suspected of bronchial asthma, for which she had medicated at a local clinic. Because she was found to have massive pleural effusion on chest radiographs, she was referred to our hospital. The effused fluid obtained by thoracocentesis was chylous. Chest and abdominal CT scans demonstrated multiple thin-walled cysts in both lung fields, retroperitoneal tumor, and protrusion on the right diaphragm and ascites. Lymphangioleiomyomatosis was confirmed by thoracoscopic biopsy. The following day after thoracic duct ligation, however, chyle leaked from the drain, and continuous intravenous octreotide infusion was instituted, which stopped the chyle leakage. Because chylous ascites increased, cell-free concentrated ascites reinfusion therapy (CART) was instituted, and chylous ascites could be maintained with good management. Therefore we should include continuous intravenous octreotide infusion and CART as attractive treatments for controlling intractable chylothorax and chylous ascites.