Objective:To investigate the clinical and pathological features of Type Ⅱenteropathy-associated T-cel lymphoma(EATL),discuss the diagnosis and differential diagnosis. Methods : One case of type Ⅱ EATL was reported with review of the literatures.The clinic symptoms,pathological morphology,immunophenotype,therapy and prognosis were analyzed in this disease. Results:A 55 years old female was admit ed with the complaint of abdominal mass.The patient always presented with chronic diarrhea,abdominal pain and fever. Histological y, the tumor cel s were monomorphic medium-sized and infiltrated the ful-thick small intestinal wal . Immunohistochemical staining, the tumor cel s expressed CD3,CD43 and CD56.The marks of CD20,CD79αand LMP-1 were negative.In-situ hybridization of EBER was negative.During the fol ow-up,patient died within 10 months.Conclusions:The diagnosis of typeⅡEATL should perform according to clinical presentations.In especial, a definite diagnosis must be on the basis of pathologic features and immunohistochemistry.