Problem statement: Ramsay Hunt syndrome is characterized by peripheral facial palsy and eruptions in external ear reportedly due to the reactivation of latent varicela zoster virus in the sensory ganglia of facial nerve. Our purpose is to describe a case of this syndrome accompanied by the Neurology Service of Nova Iguacu General Hospital. Approach: A 60-year old female patient sought the emergency room due to a complaint because she was not able to close her left eye, followed by a drift of the labial fold to the right as well as a sensation of "burning eyes" for the last three days. She also referred vertigo and bilateral hypoacusis, more intensely felt on the left side. Results: Physical examination showed a left facial palsy with a vesicular eruption in the left external auditory canal, ear lobe and neck on that side. Her taste sensation was decreased on the anterior 2/3 of the tongue, a negative rinne test, a positive weber test indicating a neurossensorial hearing loss, ataxia in walking, a Romberg sign and an abnormal fukuda pace test, however the CT scan was normal. Conclusion:The diagnosis is basically clinical, in turn, treatment is controversial. In addition to clinical findings, the diagnosis is confirmed by the presence of viral DNA in the involved tissue and vesicular exudate, as assessed by polymerase chain reaction. Ramsay Hunt syndrome involves severe dysfunction, with poorer facial nerve prognosis than in Bell's palsy. Some studies suggest that treatment with prednisone and acyclovir may improve outcome, although a prospective randomised treatment trial remains to be undertaken.