Objective To explorethe chest x-ray and MSCT findings and the diagnostic value of pulmonary lymphangioleiomyomatosis (PLAM).Methods Four cases of PLAM proven by pathology were retrospectively analyzed,and the literature was reviewed.Result The clinical manifestations were dyspnoea(4 cases),cough(2 cases),haemoptysis(2 cases),recurrent pneumothorax(2 cases)and chylous effusions (1 case).The chest radiography presented diffused reticular shadows(2 cases),honey-comb changes(1 case),pneumatothorax(2 cases),and pleural effusion(1 case).The characteristic MSCT manifestations of 4 cases with PLAM were multiple different-sized and thin-walled cystoid transparent areas, which were diffusely-distributed throughout both lungs,most cysts were of 3-15 Him in diameter.One case combined with extensive pulmonary interstitial fibrosis,pleural thickening and adhesion.Conclusions PLAM is a kind of exceptional chronic diffuse lung interstitial disease.The chest radiography has been lack of characteristic imagines,but MSCT has characteristics of PLAM,and is more valuable for accurate clinical diagnosis of PLAM.