Intravascular lymphoma (IVL), a rare, extranodal form of non-Hodgkin lymphoma (NHL), can cause infarcts by occluding small arteries of the brain. Owing to its heterogeneous clinical manifestations and unfavorable prognosis, the diagnosis is often made postmortem. Patients can present with a range of motor or sensory deficits, rapid cognitive impairment, aphasia or signs of myelitis, often with nonspecific radiographic findings.Timely recognition of a treatable disease is essential to prevent permanent neurocognitive defects. We present a case who could be treated successfully with chemotherapy.