Lymphangioleiomyomatosis (LAM) is a rare, progressive systemic disease of unknown etiology, insidious onset, often fatal, and underdiagnosed. It is exclusively found among women. LAM mainly involves the lungs where, as its name suggests, lymphatic (lymph), blood vessel (angio), and airways are surrounded by smooth muscle (leiomyoma) proliferation. It may be associated with tuberous sclerosis with clinical manifestations varying from simple cough to the development of recurrent pneumothoraces, haemoptysis, and pleural effusions. There is currently no treatment or cure. We present a rare case of a 41-year-old female, who presented with recurrent pneumothoraces, whose high-resolution computed tomography findings that were suggestive of multiple bullous lung disease, and the thoracoscopic biopsy revealed features of LAM. She was offered video-assisted thoracoscopic surgery in form of bullectomy and pleurodesis to reduce the volume of abnormal tissue and treated with progesterone hormonal therapy. The present case aims to focus on clinical-radiologic and pathologic findings and the treatment modalities available in developing countries like India where lung transplantation is seldom performed.