Background: Rheumatoid arthritis is an inflammatory disease that involves small and middle size vessels, skin, and peripheral nervous system. Cerebral involvement in the setting of rheumatoid arthritis is rare but delay in treatment will lead to high morbidity and mortality.Case Report: A 65 year old man with a history of rheumatoid arthritis and chronic consumption of prednisolone 10mg/day since 10 years ago presented with drowsiness and decreased level of consciousness following a generalized pulsatile headache and was admitted in intensive care unit and neurology consult was requested. His initial examination revealed right sided hemiparesis and central facial palsy. In spite of starting antiplatelet and anticoagulant regimen, patient’s neurological signs were exacerbated and after 72 hours he developed quadriplegia, horizontal gaze palsy and disorientation. Considering his history of collagen vascular disease and no evident of cardiovascular risk factors, a methylprednisolone pulse therapy was started with suspect to a cerebral vasculitis. After the third dose, lateralized deficits were decreased and eye movement improved. After ninth day his consciousness reached to normal level. Probable diagnosis of cerebral vasculitis was confirmed by finding a characteristic change in vessels on brain CT angiography.Conclusion: It seems that central nervous system involvement is a rare finding in rheumatoid arteritis. But in the setting of a collagen-vascular disease, development of encephalopathy and rapid progression of focal neurological deficits, when other risk factors of atherosclerosis are absent, cerebral vasculitis should always be considered. Regarding to the high morbidity and mortality, it is important to start corticosteroid pulse therapy as soon as possible after other etiologies such as infection and vascular accidents were ruled out.