Lymphangioleiomyomatosis (LAM) is a rare progressive cystic lung disease of uncertain etiology that affects young women. It appears to involve one of the most bizarre pathogenic mechanisms in human disease: the metastasis of histologically benign cells. Pneumothorax is one of the complications and most common pulmonary manifestations of LAM. We present a case of a 49-year-old Filipino, female, non-smoker and no comorbidities with recurrent pneumothorax, which was diagnosed to be a sporadic LAM. Diagnostic procedures like chest x-ray, Chest CT scan, and tissue biopsy were done. Video Assisted Thoracoscopic Surgery (VATS) and pleurodesis were done and was maintained on Sirolimus, which is the only drug proven to stabilize lung function. Pneumothorax although common may have very rare associations.