[en] The diagnostic approach, clinical evolution, and treatment of a patient with primary pulmonary lymphangioleiomyomatosis are reported. This patient presented a restrictive respiratory syndrome resistant to conventional glucocorticoid therapy. The diagnosis, based on clinical and histologic examinations, was confirmed by immunohistochemical localization of one of the desmins, the smooth muscle cell actin, and HMB45 antigen. The patient received treatment with an anti-estrogenic agent (tamoxifen citrate) and high doses of medroxyprogesterone acetate, an antigonadotropic progestin. Respiratory function improved rapidly with clinical relief.