Lymphangioleiomyomatosis (LAM) is a rare disease that occurs predominantly in females between the ages of 30 and 50 years and is clinically characterized by progressive dyspnoea on exertion, recurrent pneumothoraces, abdominal and thoracic lymphadenopathy, as well tumors-like angiomyolipomas and lymphangiomyomas. We present the case of a 42-year-old woman, who developed recurrent pneumothoraces and was subsequently diagnosed with LAM. Although pneumothorax is a common complication of the disease, its optimal approach to treatment and prevention remains unclear. Chemical or surgical pleurodesis are often performed in order to prevent recurrence, but may predispose to perioperative complications in the event of future lung transplantation.