Here, we report a case of central nervous system vasculitis in rheumatoid arthritis (RA) treated successfully with corticosteroid and mycophenolate. A 53-year-old female with poorly controlled RA presented with unilateral lower motor neurone type seventh cranial nerve palsy. Magnetic resonance imaging (MRI) of the brain revealed bilateral signal intensity changes in posterior fossa, basal ganglia, and periventricular white matter with contrast enhancement. Rheumatoid factor, anti-cyclic citrullinated peptides, and C-reactive protein were high. Cerebrospinal fluid analysis showed pleocytosis with elevated protein. Digital subtraction angiography of the brain revealed a beading pattern in distal branches of anterior, middle, and posterior cerebral arteries bilaterally suggestive of central nervous system vasculitis. We treated the patient with intravenous methylprednisolone followed by oral prednisolone tapered off in 6 months. Mycophenolate mofetil was started at the dose of 2 g per day, along with prednisolone. Sulfasalazine and hydroxychloroquine were also started to treat her arthritis. The patient showed complete neurological improvement, along with a significant resolution of the lesions in MRI.