Primary malignant lymphoma of the central nervous system (CNS) is rare and only infrequently involves the spinal cord, usually in association with multifocal CNS spread. We report a case of primary lymphoma infiltrating lumbar nerve roots without evidence of systemic lymphoma. There has been an excellent response to combined modality therapy, comprising radiotherapy with intrathecal and intravenous cytotoxics. The patient remains well and active 22 months after diagnosis. The literature regarding these rare tumors is reviewed.