An elderly patient with watery diarrhea for 3 months received extensive laboratory, radiographic and upper and lower gastrointestinal (GI) endoscopic work up including colonic biopsies, but a diagnosis was not established before death. At autopsy enteropathy associated T-cell lymphoma-type II (EATL-II) with multifocal mucosal involvement of the jejunum was identified. The colon was completely uninvolved grossly and microscopically. The stomach showed only subtle lesions grossly but microscopic examination revealed involvement by lymphoma in the stomach as well as other organs in abdomen and chest. The relationship between celiac disease and enteropathy associated T-cell lymphoma, the practical difficulties in establishing the diagnosis and the pathology of T-cell lymphomas affecting the gastrointestinal tract are discussed.