Lymphangioleiomyomatosis is a rare disease which afflicts young women of childbearing age. We experienced a 32-year-old female who was admitted because of worsening exertional dyspnea after hormonal therapy with Clomifen for five months and intermittent hemoptysis. Chest PA showed diffuse ground glass appearance with some reticular infiltrations. High resolution CT scan showed multiple small thin walled cysts distributed homogeneously throughout the entire lung. Pulmonary function test showed characteristic obstructive pattern despite the restrictive interstitial pattern of chest radiography. Thoracoscopic lung biopsy specimen showed abnormal proliferation of smooth muscle cells in the walls of lymphatic vessels, bronchioles, and pulmonary vessels typical of pulmonary lymphangioleiomyomatosis. Hormonal therapy with medroxyprogesterone was initiated.