Objective To study the clinicopathological characteristic of endometrial large cell neuroendocrine carcinoma and to emphasize its diagnosis and differential diagnosis. Methods The clinical and pathological features,immunohistochemical findings and histological diagnosis were analyzed in 2 cases of endometrial large cell neuroendocrine carcinoma(ELCNEC),with review of relevant literatures. Results Two patients aged 87 and 44 years respectively were treated due to abnormal vaginal bleeding and radical surgery were performed. All specimens showed a polypoid tumor.Microscopically,tumor of case 1 were composed of nest or cord-like distribution,focal area was microduct-like and rosettes structures was observed.Tumor of case 2 were composed of diffuse blasts with large size,scant cytoplasm,irregular nuclear contours,dispersed soot-like nuclear chromatin and showed primarily group-like distribution.Immunophenotypically,the tumor cells of case 1 were positive for CgA、CK7、AE1/AE3、EMA、P16、P53、ER、PR，focally positive for CD56、Syn,negative for WT1、CD10 and NSE.The tumor cells of case 2 were positive for CK7、Syn、NSE，focally positive for CD56、P53,negative for ER、AE1/AE3、EMA、CD10、WT1. Conclusion Endometrial large cell neuroendocrine carcinoma is a rare tumor,with a high invasiveness and poor prognosis.It may also occur simultaneously with other types of cancer.