A 33 year old woman with tuberous sclerosis and pulmonary lymphangiomyomatosis was undertaken bilateral sequential lung transplantation. Due to the progressive dyspnea and recurrent pneumothorax, she could not handle her normal daily life without oxygen supplements. The natural history of a patient with tuberous sclerosis and symptomatic lung involvement says the mean expected survival is 4 years. Such guidelines as severe obstructive lung disease, unavailable medical therapy, substantial limitation in activities of daily living, and limited life expectancy made her be a recipient of lung transplantation expecting both prolongation of life and the better quality of life. In this transplantation case, we have experienced such delicate problems as gross ventilation-perfusion mismatch, reperfusion pulmonary edema, much decreased renal function, etc.