The clinical presentation of intravascular lymphoma (IVL) is generally nonspecific and misleading and its course from onset to death is rapid. We report a case in a 70-year-old right-handed man who presented after 1 month of numbness and progressive paresis in both lower limbs and 20 days of urinary and bowel involvement. MRI of the cervical and lumbar spine with contrast failed to show signal change in the spinal cord. But brain MRI with contrast showed patchy lesions in the subcortical white matter of bilateral centrum semiovale and periventricular area. The patient's condition worsened rapidly. He died 3 months after the first occurrence of symptoms. Autopsy revealed intravascular large B-cell lymphoma. Our report indicates that myelopathy can be the initial manifestation of this disease. Brain biopsy is indicated when neurological deterioration with abnormalities is progressive in cerebral MR imaging and when tumor is suspected.