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Varicella-zoster virus (VZV) can cause variable disease states in individuals with intact and compromised immune systems. Both meningoencephalitis and thrombotic thrombocytopenic purpura (TTP) are uncommon, life-threatening entities associated with VZV. There are few reported cases of TTP due to VZV and this may be the first case of TTP due to VZV meningoencephalitis confirmed through lumbar puncture. The literature tends to emphasize that this pathology mostly occurs in immunocompromised hosts. Here, we present a unique case of TTP due to VZV meningoencephalitis in a patient that was immunocompetent.