We report a case of an immunocompetent man who developed primary spinal intramedullary malignant lymphoma. This condition occurs in about 0.9 p. 100 of primary central nervous system non-Hodgkin lymphomas in non-AIDS patients. Magnetic resonance imaging was nonspecific but suggestive. Like the brain localization, prognosis is poor. Because of the high frequency of recurrence, usually confined to the central nervous system with neuraxis dissemination, treatment must be delivered to the entire neuraxis. But more effective treatment strategies with radiotherapy-chemotherapy combinations will be needed. Feasibility and toxicity patterns remain to be determined.