A case of lymphangiomyomatosis with marked chylous ascites and persistently elevated CA-125 levels is presented. A 30-year-old woman revealed milky urine, milky ascites and exertional dyspnea about one year after the normal delivery of her second child. The lungs showed bilateral diffuse reticular shadows. No pleural effusion was observed. Pulmonary function tests revealed hypoxemia and obstructive changes of the respiratory tract. The levels of CA-125 in blood and ascites were continuously more than 200 U/ml. The inguinal lymph node biopsy and open lung biopsy confirmed the diagnosis of lymphangiomyomatosis. The patient was first treated with progesterone without effect. Oophorectomy was then performed, resulting in the improvement of respiratory distress, although ascites continued. With addition of Le Veen's peritoneojugular shunt the patient improved with reduction of ascites.