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We describe a patient with Ramsay Hunt syndrome and varicella-zoster virus encephalitis. The coexistence of these conditions is rare and to our knowledge has not been clearly documented in the English-language literature. We summarize the clinical characteristics of our patient and seven similar patients described in previous reports, including those published in Japanese. Although concomitant diseases such as diabetes and chronic renal failure may lead to an aggressive course, all patients described in detail have had good outcomes.