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Objective To study the imaging findings of the pulmonary lymphangioleiomyomatosis(PLAM), and further enhance the understanding of image, clinical, pathology, treatment and prognosis of PLAM.Methods 2 cases patients of pathologically confirmed as lymphangioleiomyomatosis were analysed with clinical, pathological, imaging performance and the related literature were reviewed.Results There were two women of reproductiveage with PLAM, the clinical features of the patients was progressive dyspnea,recurrent pneumothorax, chylothorax, hemoptysis, ascites, encreasing and disordering of the lung markings in chest X-ray.The small cystic translucent shadow was found widely distributed in the lungs for one case ; CT showed diffuse distribution of the cystic translucent shadowon with the inequality of size, with no significant difference in the distribution.The cystic cavity had a clear edge and size of less than 20mm in diameter,with 1-2mm of the wall thickness.There were showed swollen lymph nodes in the anterior mediastinal lesions.MPR post-processing can display more intuitive changes.The basic pathological features was proliferation of smooth muscle cells in the lymphatic vessels, small blood vessels around the respiratory bronchioles, causing lumen narrow or partially blocked, resulting in a series of clinical symptoms.Conclusion Lymphangioleiomyomatosis is a rare diseases of the smooth muscle cell proliferation, occurring in the whole body, mainly onset of chest and abdomen, with typical imaging and clinical manifestations.hormone therapy can be mainly used, with kown poorly clinical prognosis and with no radical cure.PLAM should be further attracted our attention.