Pulmonary lymphangioleiomyomatosis is a chronic devastating disorder afflicting women of childbearing age, characterized by proliferation of atypical smooth muscle in the lung. Attempts to treat this disease have shown that a number of hormonal manipulations may be helpful but that surgical oophorectomy alone or associated with administration of progesterone is the most effective treatment. This report describes the response to treatment with an analog of luteinizing-hormone-releasing hormone, goserelin, which is able to induce a marked suppression of the secretion of ovarian sex steroid and demonstrates with pulmonary function studies, arterial blood gas determinations, and bronchoalveolar lavage parameters that this patient's disease was responsive to this agent.