Lymphangioleiomyomatosis (LAM) is a rare progressive cystic lung disease, most prevalent in women, which affects the lung function, axial lymphatics and causes angiomyolipomas. Progressive dyspnea, pneumothorax, chylous effusions and cough are some of the clinical manifestations. It is a disease that can be difficult to manage and can have a poor prognosis, especially if not promptly addressed. We present a case report of a 43-year-old woman who was admitted in 2014 with recurrent chylothorax

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... d diagnosed with LAM. However, since the actual best therapeutic approach, sirolimus, was not yet recommended at the time, together with the patient's low adherence to medical follow-up, her clinical management was difficult with some complications. After her return into medical care, the disease had progressed substantially; however, it stabilized after starting sirolimus therapy. This case report aims to exemplify how the management of this disease can be difficult and highlight sirolimus as the "weapon" to improve its prognosis even in an advanced stage of the disease.