Rheumatoid arthritis may cause central nervous system complications by means of various mechanisms. We describe the case of a patient with a new diagnosis of rheumatoid arthritis (RA) complicated by normal-pressure hydrocephalus. After treatment with prednisone, the patient improved remarkably as regards mental status, urinary control and gait. We suggest that normal-pressure hydrocephalus may occur as an extra-articular manifestation of RA, caused by a pathogenic inflammatory mechanism. We analyse previous case reports describing a relationship between RA and normal-pressure hydrocephalus, and discuss potential mechanisms underlying this association.