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Abstract Background and purpose Optimal treatment of intracranial stenoses in varicella zoster virus (VZV)-associated vasculitis is unknown. This study aims to evaluate the merits and potential pitfalls of a specific therapeutic strategy, initially proposed by Don Gilden in 2015. Methods We describe three patients with intracranial stenoses caused by VZV vasculitis successfully treated by a long-term combination of valacyclovir and prednisolone. Results All three patients were young men suffering from stroke. Only one reported a first contact to VZV in adulthood. All three presented stenoses in the intracranial part of the internal carotid artery or the proximal segments of the middle cerebral artery as well as an elevated cell count and positive VZV antibody index in cerebrospinal fluid. They received a combination therapy regimen with prednisone and valacyclovir about a minimum of one year. Intracranial stenoses improved markedly in one and almost resolved completely in the other two patients. Side effects of corticosteroid treatment occurred in two patients. Conclusions Long-term combination treatment with prednisone and valacyclovir proved to be effective in three young men suffering from intracranial stenosis due to VZV vasculitis.