The authors report on the case of a 64-year-old man with solitary intradural extramedullary non-Hodgkin lymphoma of the cervical spine. The lesion mimicked the appearance of meningioma on MR imaging. Positron emission tomography showed increased accumulation of fluorine-18–labeled fluorodeoxyglucose only in the cervical lesion. Serum levels of C-reactive protein and soluble interleukin-2 receptor were mildly elevated. At surgery, the intradural tumor in the subarachnoid space was totally extirpated. Based on histopathological findings, diffuse, large B-cell type non-Hodgkin lymphoma was diagnosed. Postoperatively, the patient was treated with 2 courses of chemotherapy by intrathecal injection of methotrexate, cytarabine, and prednisolone and 4 courses of intravenous rituximab, an antibody binding to CD20 on the surface of B cells. All preoperative symptoms completely resolved after surgery. Two years postoperatively, the patient was faring well with no evidence of local recurrence or new lesions at any other site. To the best of the authors' knowledge, this case is the first reported instance of solitary intradural extramedullary non-Hodgkin lymphoma of the cervical spine.