Lymphangioleiomyomatosis (LAM) is a rare lung disease that affects young women. It involves the abnormal growth of smooth muscle cells in the lungs and lymphatic vessels. A 34-year old female patient underwent a lung resection for suspected LAM. Histological examination revealed features consistent with LAM, including cystic lung destruction. LAM is often misdiagnosed and requires differentiation from other cystic lung diseases. While there is no effective treatment, mTOR inhibition shows promise in stabilizing lung function and symptoms in LAM patients.