Extranodal presentation in non-Hodgkin's lymphoma (NHL) is uncommon, and the mandible is very rarely involved. Primary NHL of the mandible, for the most part, has intermediate or high malignancy and has a much greater incidence of local recurrence compared with other sites of involvement. A 48-year-old Japanese man with NHL of the mandible received radiotherapy, followed by high-dose chemotherapy supported with peripheral blood stem cell transplantation (PBSCT). High-dose cyclophosphamide, Adriamycin, and vincristine were used for pretransplant conditioning. He achieved complete remission and has survived in continuous complete remission for more than 72 months to date. Marrow-ablative chemotherapy facilitated by PBSCT is thought to be useful as part of the primary therapy for patients with NHL who have poorer prognoses.