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Non-Hodgkin lymphoma (NHL) of the ileum, presenting as perforation and peritonitis, is a rare disease, derived from intestinal intraepithelial T lymphocytes. The degree of malignancy is extremely high. The pathogenesis of ileal perforation caused by NHL remains unclear, as well as the chromosome and immune system abnormalities, which may be related to NHL, and are indistinguishable from other benign and malignant conditions and are clinically nonspecific.