BACKGROUND Approximately 10% of patients with peripheral facial nerve palsy are children. Facial nerve palsy is usually idiopathic. An uncommon cause is herpes zoster oticus (Ramsay Hunt syndrome). This syndrome usually affects adults and is rare in children. METHODS We reviewed the literature and describe the cause, clinical manifestations, and treatment of Ramsay Hunt syndrome. We also report a case of the syndrome in a 12-year-old boy. CONCLUSIONS Ramsay Hunt syndrome is characterized by peripheral facial paralysis associated with herpetic eruption on the auricula and external ear, and by vestibulocochlear dysfunction. It occurs by reactivation of latent varicella-zoster virus (VZV) in the geniculate ganglion, affecting the seventh and eighth cranial nerves. The diagnosis is based on history and physical findings. Treatment of Ramsay Hunt syndrome uses a combination of high-dose corticosteroids and acyclovir. Although the prognosis is better in children than in adults, it is not good enough.