A 63-year-old African-American man with multiple small intestinal lesions presented with intestinal perforation. Segmental excision of the small intestinal lesion showed transmural infiltration of small to medium size lymphoid cells arranged in sheets with splaying and destruction of submucosal glands. The cells showed folded, irregular nuclear contour. Frequent apoptotic bodies were seen. Immunohistochemical stains showed that the lymphoid cells were positive for CD3, EBER (diffusely positive) and negative for CD20, CD5, CD7, CD8, CD30, CD10, ALK1, PAX5, cyclin D1, CD21 and S100. The proliferation index (Ki67) was high at 95%. PCR testing for T-cell clonality gene rearrangement showed monoclonal population of tumor cells.