We report on four patients with a histologically proven diagnosis of arteritis temporalis and clinical and/or neuroradiological evidence of severe focal cerebral ischemia due to intracranial vasculitis. While one patient suffered from a transient ischemic attack, CCT and MRI scans of the other patients showed multiple lacunar infarctions, combined with territorial infarctions in two cases. Necropsy in one patient demonstrated generalized giant cell vasculitis in large and small cerebral vessels. We suppose that the cerebral involvement was provoked by insufficient steroid therapy of arteritis temporalis in two patients. In one case, remission could be achieved by a combination of high-dose steroids and cyclophosphamide; one further patient remitted under lower steroid dosage. Steroid therapy was ineffective in two patients, one of whom died due to secondary complications. We conclude that central nervous system affection is a rare but dangerous complication of arteritis temporalis and may present as cerebral micro- and macroangiopathy.