Palatal myoclonus is an extremely rare disorder. Tinnitus is secondary to rhythmic involuntary movements of the soft palate. Clinical diagnosis is based on the confirmation of the soft palate movements synchronous with an audible clicking noise outside. In this article, we report a 38-year-old female case with a 10 year-history of continuing ear click, who was diagnosed with essential palatal myoclonus. The disease and its differential diagnosis from symptomatic palatal myoclonus were discussed in the light of literature data.