Central nervous system vasculitis is a rarely described complication of rheumatoid arthritis. We report a case of cerebral vasculitis in a 55-year-old woman with a 7- year history of seropositive, nodular rheumatoid arthritis. Striking multifocal abnormalities of the white matter on magnetic resonance imaging led to a suspicion of vasculitis despite lack of clinical evidence of extracranial vasculitis and normal findings on cerebrospinal fluid studies and cerebral angiography. After institution of treatment with glucocorticoids and azathioprine, she survived in stable condition for 14 months from the onset of symptoms. Postmortem examination of the brain revealed vasculitis and chronic ischemic changes. © 1995 Southern Medical Association.