AbstractRamsay‐Hunt syndrome, herpes zoster oticus (HZO), derived its name from James Ramsay Hunt, who first described it in 1907. It is classically characterized by acute peripheral facial paralysis, herpetic eruptions on the auricle, and vestibulocochlear dysfunction due to the reactivation of varicella zoster virus (VZV). In this Case Report, the authors describe an HZO patient with simultaneous VZV encephalitis. To date, only eight cases of HZO associated with VZV encephalitis have been reported in the English literature. Therefore, the authors discuss all the aspects of this rare entity, including clinical examination, radiological evaluation, laboratory evaluation, and treatment options. Laryngoscope, 2009