Essential myoclonus is a rare and benign, frequently hereditary disorder although it may appear sporadically. In this clinical picture, myoclonus is the only neurological sign, meanwhile a distonic component may accompany. Myoclonus affecting predominantly the arms and neck, occurs spontaneously or with action. In this study a 45 year-old woman diagnosed as essential myoclonic dystonia was presented. Her complaints had started when she was 1,5 years old, and the diagnosis was made when she was 38 years old after long term follow-up and detailed electrophysiological examinations.