We report a case of glottic primary laryngeal lymphoma. Although the head and neck region is a frequent site of origin of extranodal non-Hodgkin's lymphomas, laryngeal involvement is exceptional. Including this case, about 90 primary laryngeal lymphomas have been reported in the literature. Microscopic study showed a diffuse malignant lymphoma of high-grade malignancy (WF sub-division H). A diffuse, large, B-cell-type NHL was diagnosed histopathologically. The patient was treated with combination chemotherapy, including cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP), which resulted in complete clinical remission after two courses. Four courses of combination chemotherapy were subsequently performed, making a total of six courses of combination chemotherapy. No recurrence has been observed during the 16-month follow-up period.