Central nervous system varicella-zoster virus related vasculopathy in HIV patients can cause severe neurological syndromes. The authors identified 55 patients from case reports and series, and analyzed the clinical, laboratory, radiological, and pathological information. One third of the patients never had a varicella zoster rash. Clinical studies revealed abnormal angiograms, cerebrospinal f luid (CSF) pleocytosis, and varicella-zoster virus DNA in the CSF. Patients treated with acyclovir and corticosteroids usually had a good recovery.