A 61-year-old woman presented with acute intense lower back pain and weakness in her left leg. She also presented with throbbing headache on the same day. On admission, muscle weakness in her left leg, lower left quadrantanopia and left lower extremity deep sensory disturbance were observed. Laboratory data showed no coagulopathy and autoimmune antibody was negative. Cerebrospinal fluid examination showed bloody and inflammatory findings. Brain MRI revealed cerebral infarction with multiple intracranial arterial stenosis and convexal subarachnoid hemorrhage. Spinal MRI revealed spinal hemorrhage in the cervical, thoracic, and part of the lumbar spine. Because these lesions occurred simultaneously, we made a diagnosis of vasculitis. After high dose corticosteroids therapy was undertaken, the multiple arterial stenosis improved. Primary angiitis of the central nervous system is sometimes difficult to distinguish from reversible cerebral vasoconstriction syndrome in its initial stage; although symptoms, examination findings and treatment differ in both.