Introduction: Background The CNS inflammatory lesions in rheumatoid arthritis (RA) present as rheumatoid nodules, meningeal vasculitis and rheumatoid cerebral vasculitis, the latter being a rare and serious complication estimated in 1–8% where the blood vessel is the target of immune reaction. Most culminate in death, especially when cerebral vasculitis is associated with systemic rheumatoid vasculitis. Methods Case report. Results A female 22 yo, secretary, diagnosis of RA one month prior to treatment with NSAID, who had fever, nausea and bifrontal headache, oppressive, which increased the Valsalva maneuver. 1 week after vomiting followed by inattention. The following day she presented disorientation and incomprehensible language, with improvement of these symptoms during the rest of the day. At her entrance she presented a conjugated deviation look to the left and right hemiparesis, she went to medical assessment. Physical Examination: Awake, partially oriented, fluent language, incongruous, gnosis and praxis performed with difficulty, reasoning, abstraction and calculation altered. Cranial nerves and force are normal, generalized hyperreflexia, bilateral flexor plantar response. Palmomentonian reflex present. Cerebellum-vestibular unsteady gait, short steps, required help when performed with indistinct lateropulsion. Romber (+). Absent meningeal signs. Laboratory and other diagnostic tests: Normochromic, normocytic anemia (hemoglobin 11.6 g/dl) leukocytosis 11,000/mm3, lymphopenia 900/mm3, hypoalbuminemia 2.7 mg/dl, increased LDH 343U/L. Renal failure (proteinuria 20). Inflammation (elevated C-reactive protein 97 mg/L). Rheumatoid Factor (+) 203 UI/ml. Immunological profile C3 81 mg/dl, C4 13.2 mg/dl, rest of antibodies in range not pathological. Non-reactive HIV1/2 ELISA.VDRL, anti-cysticercus antibodies, Mycological and culture for Tb mycobacterium in CSF was negative. CSF analysis: transparent, cells 11/mm3, hypoglycemia 29 mEq/dl, proteins elevated 117 mg/dl. EEG in awake is abnormal, mild generalized dysfunction without epileptic activity. Brain MRI on contrast-T1 weighted image, coronal section with meningeal enhancement compatible with pachymeningitis. Macroscopic view of external surface of the encephalus, on the dorsal side there is slight congestion of the leptomeningeal vessels. Histopathologic pattern of brain stem, central region blood vessel with wall necrosis, in addition to inflammatory infiltrate and the presence of nuclear dust. Histological section of kidney shows blood vessel with presence of wall necrosis and fibrin deposit. Conclusion Rheumatoid cerebral vasculitis is an uncommon entity, with variable neurological manifestations, sometimes with signs of focalization or diffuse manifestations and characteristically with fluctuating evolution. The literature reports the diagnosis is made retrospectively during a postmortem histopathological study in most cases, consistent with the case presented.