A case of a 30-year-old primiparous woman with pulmonary lymphangioleiomyomatosis is described. The patient had experienced six episodes of spontaneous pneumothorax at the age of 27 years and had been diagnosed as having pulmonary lymphangioleiomyomatosis based on histological findings of specimens obtained by transbronchial biopsy. She had undertaken open lung surgery and thoracoscopy. Thereafter, she became pregnant spontaneously. Her antenatal course was uneventful with no exacerbation of respiratory status. At 38 weeks of gestation, she underwent a selective cesarean section and myomectomy under combined spinal and epidural anesthesia. Her postoperative course was uneventful. No remarkable changes in computed tomographic findings of the lung were noted on the 20th day of postoperation compared with those before pregnancy. She has been followed-up in the pulmonary outpatient clinic with no deterioration of the disease.