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Mantle cell lymphoma (MCL) is a type of non-Hodgkin lymphoma that affects mature B cells. MCL has been found to have extranodal involvement in the bone marrow, spleen, liver, and gastrointestinal tract. It is most commonly associated with lymphadenopathy and hepatosplenomegaly, but a leukemic presentation is unusual. Colon cancer and mantle cell lymphoma coexisting in the same gut is unusual. This study has been submitted as a case report for publication. After being diagnosed with recurrent mantle cell lymphoma, a 45-year-old male patient was admitted for the third cycle of treatment. A colonoscopy revealed mantle cell lymphoma, which had previously been treated with chemotherapy and now appears to be active in the ileocecal region. A biopsy was performed for further investigation. Histopathology reveals small intestine mantle cell lymphoma, and the patient is currently undergoing chemotherapy. After the third cycle of chemotherapy was planned with medical management, the patient was referred for oncologic treatment. We present a case of MCL with large nodules in the last 5 cm of the ileum, as well as the workup of this uncommon lymphoma, which clinicians may be asked to detect and manage. This report serves as a reminder to maintain a broad differential to account for uncommon diseases and pathology. Practising with a thorough understanding of medical principles and clinical acumen is critical for optimal patient care and, as demonstrated in this case, avoiding a potential surgical intervention that would delay appropriate chemotherapy.

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