Lymphangioleiomyomatosis (LAM) is a rare pulmonary disease of unknown etiology, which affects exclusively the young woman. LAM is associated with much morbidity and can lead to respiratory failure and death unless lung transplantation is performed. The proliferation of abnormal smooth muscle cells (LAM cell) in the lungs, causes destruction of the cystic parenchyma and chronic respiratory failure. The main manifestations are respiratory, revealed by pneumothorax and or chylothorax. The definitive diagnosis is based on the lung biopsy, but in the absence of the latter, criteria diagnostics have been proposed based mainly on the CT aspect, and the presence of renal angiomyolipoma's. The treatment is mainly symptomatic. Sirolimus appears to stabilize respiratory function and improve symptoms, while hormonal treatment is in progress evaluation. However, transplantation remains the only validated treatment. We report two cases of two women with LAM, with different circumstances of discovery and evolution.