We reported a case of aortitis syndrome in a 65-year-old female patient who had been suffering from chronic intense pain of occipital region, nape and left shoulder, and ataxia for about two years. The symptom was not controlled by the administration of NSAIDs, anticoagulant drug, platelete aggregation inhibitor, cerebral metabolic enhancer and so on. Stenosis of left vertebral artery was revealed by neck MRA, and blood hypoperfusion of cerebellum and left occipital lobe was disclosed with brain perfusion scintigraphy. The symptom was successfully treated with i. v. injection of 40μg prostaglandin E₁ and oral administration of 10mg/day prednisolone. We suspected hat blood flow effect of prostaglandin E₁ and anti inflammatory effect of steroid treatments were effective in a patient with aortitis syndrome.