Lymphangioleiomyomatosis (LAM) is a rare disease of unknown origin that affects women of reproductive age. The presence of spontaneous pneumothorax, hemoptysis, and chylous effusion or ascites in a young woman prompts the diagnosis. Most patients present with progressive dyspnea and cough. High-resolution CT usually shows bilateral diffuse cystic airspace disease with cysts of variable sizes. Pulmonary function tests show predominantly an obstructive pattern. The treatments available for LAM at this point are not clearly effective. Since its description more than 50 years ago, various treatment modalities including corticosteroids and cytotoxic agents have been tried without any significant benefit. Because the disease predominantly affects women of childbearing age, hormonal manipulation has been attempted with questionable success. Recent advances in molecular biology have thrown some light on the pathophysiology of this disease and may pave the way for future therapies. Most patients who have LAM die within 10 to 15 years of diagnosis because of progressive respiratory failure. More than 40 patients with LAM have had lung transplantation. LAM-specific posttransplant complications occur, and there are rare reports of recurrence of the disease in the transplanted lung. In this review, we describe the clinical, physiologic, radiologic, and pathologic characteristics of this disease and discuss the various currently available treatment options as well as future possibilities in the treatment of this rare and progressive disease.