Primary gastrointestinal Lymphoma is a relatively infrequent tumor, who could be effectively treated with both surgery and chemotherapy or radiotherapy. The design of treatment depends on prognostic factors, namely stage and histologic grade. The most functional staging system seems that of Mushoff, while the recent concept of "Mucosa-associated lymphoid tissue" has allowed to correlate the histologic grading with prognosis. Surgery is the most used therapeutic approach in IE and IIE stages, but both chemotherapy and radiotherapy or their combination seem to have similar activity. Adjuvant chemo- and/or radio-therapy after surgery increases survival compared to surgery alone. Chemotherapy is the treatment of choice in more advanced stages.