Pulmonary lymphangioleiomyomatosis is an extremely rare interstitial lung disease. The clinical presentation is generally recurrent spontaneous pneumothorax and progressive dyspnea. No definitive treatment option is available and it leads to respiratory failure due to multicystic destruction of the lung parenchyma in the following years. A 39-year-old female patient was admitted to our clinic with the complaints of sudden onset dyspnea and diffuse chest pain. Auscultation revealed decreased respiratory sounds in both lungs. Chest X-ray showed bilateral pneumothorax and bilaterally tube thoracostomy was performed. Thoracic tomography demonstrated a few thin-wall cystic structures in both lungs with a higher number on the right side. We performed bullectomy, apical wedge resection, apical parietal pleurectomy and mechanic pleural abrasion on residual pleural spaces through right posterolateral thoracotomy. One month later, we performed bullectomy, bulla ligation, apical wedge resection, apical parietal pleurectomy and mechanic pleural abrasion on residual pleural spaces through left posterolateral thoracotomy due to the left recurrent pneumothorax. The patient was diagnosed with lymphangioleiomyomatosis based on the histopathological examination. No recurrent pneumothorax was observed at the end of the two-year follow-up period. Lymphangioleiomyomatosis should be kept in mind in premenopausal women who have bilaterally recurrent spontaneous pneumothorax. Early surgical treatment should be planned in these patients due to multiple pneumothorax recurrences with conservative procedures.