A 40 years-old woman with a previous history of a right pneumothorax in 2013 had a left pneumothorax with persistent air leak in 2020. The computerized tomography scan of the chest described diffuse emphysematous changes with multiple "small bullae" in the upper lobes. She was submitted to mechanical and chemical pleurodesis with a wedge apical resection by uniportal video-assisted thoracic surgery. Pathological findings confirm the diagnosis of pulmonary lymphangioleiomyomatosis.