A 71-year-old woman with active rheumatoid arthritis (RA) was referred to our department because of multiple intracranial nodules. On admission, the RA disease activity was very high even after the treatment of methotrexate in other hospital. She underwent open biopsy to confirm a histopathological diagnosis of the intracranial lesions. Surgical specimen mainly consisted of necrosis surrounded by epithelioid cells. The masses were reduced spontaneously in size without additional treatment. Eleven month later, the lesions were relapsed. She underwent treatment with corticosteroid, and the lesions were remarkably regressed. The clinical course and histological examination were compatible with rheumatoid nodule (RN). Intracranial RN is extremely rare and its clinical course is not completely understood. In active RA patients, RNs should be considered, and histological diagnosis is inevitable for following suitable treatment.