Primary mantle cell lymphoma (MCL) in the gastrointestinal tract is rare, accounting for 4–9% of all reported cases of gastrointestinal non-Hodgkin lymphoma. Furthermore, involvement of the entire gastrointestinal tract in MCL is rare. The present report describes an example of MCL characterized by numerous diffuse polypoid lesions along the whole digestive tract. In particular, there was a focus on the endoscopic presentation of the digestive tract. The patient initially received a treatment regimen of rituximab combined with cyclophosphamide, doxorubicin, vincristine and prednisone. After two cycles of treatment, the regimen was changed to rituximab combined with etoposide, oxaliplatin and ifosfamide, with the addition of ibrutinib capsules. Patients with MCL have a poor prognosis; however, complete response can be achieved after treatment.