Purpose To study the clinicopathological features of lymphangiomyomatosis (LAM) of pelvis lymph node.Methods A patient with endometrial endometrioid adenocarcinoma and LAM was analyzed including clinical data and pathological features.HE and immunohistoehemistry of EnVision stainings were used,and the literatures were reviewed.Results Well-moderately differentiated endometrioid adenocarcinoma could be observed in the endometrium.Proloferation of LAM cells were seen in the capsule and medulla of the pelvic lymph node.The LAM cell was spindle,epitheliod and polygonal cells with oxyphilic or clear cytoplasm which arranged surrounding lacunes.The LAM cells showed no atypia and mitosis could not seen.The tumor cells showed diffusely positive for SMA,Caldesmon,desmin,vimentin,ER and PR,the cells lining the lacunes were positive for CD34 and D2-40.The epitheliod cells were positive for HMB-45 and negative for Melan-A.The Ki-67 immunostaining showed a proliferation index of ＜ 1％.Conclusion LAM is an uncommon neoplastic multisystem disease that affects the lungs mostly.Endometrial endometrioid adenocarcinoma with LAM of pelvic lymph node is extremely rare.The diagnosis can be made according to the histological characteristics and immunohistochemical features.Moreover this conclusion will provide the clinicopathological materials for the future study about LAM.