Pulmonary lymphangioleiomyomatosis is a rare disease occurring almost exclusively in woman of reproductive age. It is characterized by rapid deterioration of respiratory functions and results in death within an average of 10 years. This disease is caused by a progressive proliferation of atypical smooth muscle in the pulmonary lymphatic vessels, blood vessels, and airways leading to the development of distal cystic changes which causes frequent recurrent pneumothoraces. We experienced a case of pulmonary lymphangioleiomyomatosis in a 30-year old woman who had a history of bilateral, recurrent pneumothoraces. The patient underwent lung biopsy through right thoracotomy which revealed the diagnosis of pulmonary lymphangioleiomyomatosis. We report a case with a review of the literature.