Pulmonary lymphangioleiomyomatosis (LAM) is a rare and progressive disease of young women that usually causes death from respiratory failure. Here we report an early case that was diagnosed by thoracoscopy. A 28-year-old woman presented to a local clinic with chest pain and her chest X-ray film showed left pneumothorax. After placement of a chest tube, the lung re-expanded fully. Following the recurrence of left pneumothorax, she was referred to our hospital and underwent video-assisted thoracoscopic surgery (VATS). However, left pneumothorax recurred again one month later. She underwent a second thoracoscopic operation, during which a bulla was recognized in the lingular segment of the left lung and was resected. On histological examination of the surgical specimen findings consistent with LAM were obtained. Taking the preoperative imaging findings and the thoracoscopic findings into consideration, a diagnosis of early lymphangioleiomyomatosis was made. Thoracoscopic management of pneumothorax in young women can facilitate the early diagnosis of this condition.