Lymphangioleiomyomatosis, a multisystem disease characterized by cystic lung lesions can result in respiratory failure and is considered to be sex hormones related. No effective treatment for lymphangioleiomyomatosis is currently available. We report a 35-year-old patient in her second pregnancy. She also had experienced five episodes of spontaneous pneumothorax at the age of 30. Despite excessive estrogen production during pregnancy she had mild non-progressive exertional dyspnea without limitation of daily activities during pregnancy without deterioration of respiratory status.