Pulmonary lymphangiomyomatosis is a rare disease of smooth muscle proliferation in the walls of lymphatic vessels and in the interstitial areas of the lungs. It only affects women of reproductive age. The majority of patients die from respiratory failure within 10 years. A new observation of pulmonary lymphangiomyomatosis is reported in a woman of 36 years. The disease was discovered at an advanced stage of respiratory failure. Left-sided pleurectomy and treatment with medroxy-progesterone and tamoxifen did not improve the patient's condition.