Primary spinal lymphoma carries a low incidence and poor prognosis. The most reliable diagnostic signs have been segmental swelling of the cord on myelography and increased cellularity of cerebrospinal fluid. Biopsies of spinal tumors or their cerebral extensions showed lymphoma of the non-Hodgkin type only. Peroxidase-antiperoxidase staining for light chains helps to distinguish well differentiated lymphomas from infectious processes in some instances. A lymphoma in an immunosuppressed patient fulfilling all criteria for primary growth in the spinal cord is described, and the case compared with others in the literature. Combined radiotherapy and chemotherapy provided a 48-month survival in one patient.