Palatal myoclonus (PM) is a rare neurotological disorder but is being reported with increasing frequency. Two forms, symptomatic and essential PM, have been identified as separate clinical entities and probably also have separate etiologies. In essential PM, brain imaging is unremarkable and a clicking noise caused by peritubal muscle contractions and often audible by the examiner usually is the patients' chief complaint. The treatment of essential PM with systemic application of anticonvulsants and related substances as well as by destructive surgical means has yielded unsatisfactory results. Recently, the use of botulinum neurotoxin has emerged as a new treatment option that seems particularly attractive due to its safety, complete reversibility, very few side effects, and minimal invasiveness. We report the case of essential PM in a 10-year-old girl who was successfully treated with botulinum toxin injections under eletromyographic guidance and we review the literature on the use of botulinum toxin in PM.