Intestinal T-cell lymphomas are fairly uncommon, and can sometimes be associated with enteropathy. Enteropathy-associated T-cell lymphoma (EATL) is commonly accompanied by a nonspecific mucosal ulceration, similar to that observed as a complication of celiac disease. The clinical course of EATL is quite unfavorable, and tends to have a generally poor prognosis. When a tumor invades the bowel wall and is treated with corticosteroids and chemotherapy, cell lysis with perforation often occurs, particularly in case of lymphoma. Recent data indicate that extensive resection may improve local control, and eliminate the risk of early mortality due to visceral perforation or hemorrhaging in unresected lesions during chemotherapy. Here, we report the case of a 51-year-old male who was diagnosed with primary gastrointestinal lymphoma after colonoscopy, and presented with EATL after emergent exploratory laparatomy. We also include a review of the literature regarding this uncommon entity.