Ramsay Hunt syndrome (RHS) is a rare, severe complication of varicella-zoster virus reactivation in the geniculate ganglion, characterized by hearing loss, pain, and vesicles in the ear or mouth along with ipsilateral facial palsy. Although it is rare, it is more commonly found with immunodeficiency conditions. We report a case of a 35-year-old human immunodeficiency virus positive male, having CD4+ count of 336/μl, who presented with RHS and had vertigo, painful vesicular eruptions on the right ear and unilateral sensorineural hearing loss. He was treated immediately with valacyclovir and prednisolone, and the complete recovery was achieved at 6 months after the onset.