Intravascular lymphoma is a rare, extranodal, large B-cell lymphoma in which neoplastic, lymphoid cells proliferate within the lumina of small to medium-sized vessels. Neurological findings develop in about two-thirds of patients and usually present as multifocal cerebrovascular events, subacute encephalopathy, spinal cord or nerve root vascular syndromes, or peripheral and cranial neuropathies. In the brain, intravascular lymphoma manifests primarily as multiple infarcts. The strokes may involve any area of the brain, although supratentorial infarcts are more prevalent than cerebellar or brainstem infarction. Encephalopathy is observed in intravascular lymphoma patients and may be accompanied by focal neurological signs or seizures. The peripheral neuropathy observed in intravascular lymphoma is a predominantly axonal neuropathy. Computed tomography (CT) and cerebral angiography may show characteristics of stroke and vessel occlusion, in many instances, these studies are normal. Systemic chemotherapy with anthracycline-based regimens has been the most commonly pursued treatment in the literature.