Lymphangioleiomyomatosis (LAM) is a rare disease in women in childbearing age leading to progressive respiratory failure. LAM is characterized by an abnormal harmartomatous proliferation of smooth muscle cells surrounding the blood vessels, lymphatics and airways in the lung. This proliferation leads to airway obstruction, cystic alveolar change and lymphatic obstruction. Patients present with dyspnea, pneumothorax, cough, chest pain, hemoptysis, and chylous effusion. Although lung transplantation is the only therapeutic modality in end-stage LAM with respiratory failure, there has not been any report of successful treatment in Korea. We report one case of successful left single lung transplantation in a 40 year old woman suffering from end-stage LAM.