Primary laryngeal lymphoma is a very rare entity, with fewer than 50 cases reported in the English literature in the past 60 years. Close scrutiny of some of these case reports reveals that the larynx was not always the only site of involvement, thereby diminishing the total number of patients with primary laryngeal lymphoma to fewer than 35. The authors report a series of six patients, who were seen and evaluated at the Mayo Clinic between 1952 and 1995, with stage IAE non-Hodgkin's lymphoma of the larynx. Three patients had large-cell lymphomas according to the REAL (Revised European-American Lymphoid) classification. The other three had a small lymphocytic lymphoma, follicular small cleaved lymphoma, and follicular mixed lymphoma. All patients received radiation therapy alone as initial therapy for their disease and all patients had a complete remission to initial therapy. Four patients subsequently relapsed and the histology at relapse was the same as the initial histology in all four patients. Five patients have died, three of lymphoma, with a median survival of 67 months (range, 40 to 228 months). In view of the heterogeneity of histologies in this group of lymphomas, the variability in duration of response, and the significant number of patients who died of their disease, it is more likely that primary laryngeal lymphoma is an unusual presentation of non-Hodgkin's lymphoma than a separate disease entity. Despite the small number of patients in this study, the data would suggest that patients are best treated according to the histology of the lymphoma, rather than the limited stage and location of the disease.