Three cases are reported of lymphangiomyomatosis with pulmonary and abdominal manifestations. Two had a chylous pleura effusion, while the third presented a retroperitoneal manifestation, which was completely resectable. Antiestrogen therapy with tamoxifen was administered in all three cases. Two patients died of pulmonary progression after 4 months of therapy. The third is still alive, with stable disease for more than 6 years, and has been receiving tamoxifen for 66 months. These observations indicate that antiestrogen treatment may be as effective as oophorectomy when started at an early stage of the disease.