A 41-year-old male presented to our clinic with a 1-month history of left hemiparesis. He had marked left arm weakness. The diagnostic work-up revealed an intramedullary mass at spinal level C2-4. Laminectomies were performed at C2-3-4 and the tumor was subtotally resected. Histological examination identified the mass as a non-Hodgkin's diffuse B-cell lymphoma. The patient was treated with corticosteroids, chemotherapy, and adjuvant radiotherapy. The residual tumor tissue had completely disappeared by 6 months of follow-up; however, the patient presented with intraventricular metastasis at 11 months postsurgery.