Introduction: Enteropathy-associated T cell lymphoma (EATL) is a rare peripheral non-Hodgkin's T cell lymphoma originating from intraepithelial T lymphocytes of the intestines. In general, this condition has a poor prognosis. A common initial presentation of this cancer which is a small intestinal perforation necessitating emergency surgery is of interest to practicing surgeons. The diagnosis is rarely made prior to pathological examination. Methods: We report a case of a 39-year-old African American man who presented with acute abdomen and was found to have a deep necrotic ulcer of the jejunum during exploratory laparotomy. Results: Pathological examination and laboratory results demonstrated EATL, type 2, which is not associated with celiac disease. A review of the literature on EATL is also presented.
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