Varicella-zoster virus (VZV) infection is usually benign and self-limited. However, particularly in the immunosuppressed host, serious central nervous system complications may occur, including encephalitis, myelitis, and cerebral vascular occlusion. We report the case of a 57-year-old male with AIDS, who rapidly developed a sixth cranial nerve palsy and progressive myelopathy. There was no antecedent zoster rash. Autopsy revealed VZV ventriculo-encephalitis and vasculitis, as well as a transverse infarction of the spinal cord without evidence of direct infection of the cord parenchyma. Spinal cord infarction secondary to VZV vasculitis is an unusual cause of myelopathy in immunosuppressed patients.