Case Presentation: A 36-years old man presented with five weeks history of intractable diarrhea. Colonoscopy was normal, but abdominal computed tomography (CT) scan revealed mural thickening at duodenojejunal junction, and subsequent jejunofiberoscopy showed a circumferential ulceration at the jejunum. Histo-immunopathology confirmed the diagnosis of enteropathyassociated T-cell lymphoma (EATL) type II. His disease course proved to be aggressive and refractory to standard front-line chemotherapy, and eventually progressed through second-line salvage regimen with CNS and intracranial involvement. He died nine months after the initial diagnosis.