Palatal myoclonus is a rare neurological disorder of the soft palate and other oropharyngeal muscles, which causes clicking tinnitus. The latter is audible both to the patient and to an observer. The aetiology may be a brain stem lesion, and it is only rarely that a cause cannot be identified. The condition has been described in adults, but seldom in children. We present here a case of palatal myoclonus with distressing tinnitus in a 12-year-old girl and its successful treatment with electromyography (EMG)-guided injections of Clostridium botulinum toxin.