Encephalitis is the most severe manifestation of central nervous system (CNS) infection by Varicella-Zoster-Virus (VZV). VZV associated encephalitis is now recognized to be a vasculopathy that affects large or small cerebral arteries. This report describes an autopsy case of an immunocompetent 66-year-old male who developed a progressive small vessel vasculopathy and clinically presented with a zosteriform rash and myelo-radiculoganglio-meningo-encephalitis followed by subarachnoid bleeding. This is an extremely rare manifestation of VZV vasculopathy associated with widespread CNS damage, and what is more, the spinal lesions were different from those of the cerebrum, brainstem and cerebellum, where the former were predominantly demyelinative changes and the latter were ischemic. To the best of our knowledge, few cases have been described pathologically in an immunocompetent individual. Further studies are needed to investigate the pathogenesis and treatment of VZV vasculopathy.