Deep brain stimulation (DBS) has become one of the major therapy options for movement disorders including dystonia. This article should give a review of the current literature from a neurosurgical perspective. Since dystonia is a rare disease, only few studies on larger cohorts have been published, and very few randomized controlled studies are avaialable in the international literature. Our experiences gained treating 134 patients with various types of dystonia, between 1999 and 2015, will serve a guide to interpret the current literature. Symptoms of dystonia are due to a variety of medical conditions. A careful and extensive neurological evaluation is mandatory before medical and surgical treatment options are considered, since the clinical benefits of more aggressive treatment e.g. by DBS depend to a large extent on the etiology of the disease. Diagnostic steps should include also magnetic resonance imaging (MRI) and possibly genetic evaluation. Therapy consists of physiotherapy, medical therapy including botulinum toxin injections in focal dystonia and DBS. This neurosurgical therapy is considered a highly effective therapy in well selected patients, which should be discussed, depending on the etiology, early in the patient's career. Patients with primary dystonia will benefit the most from DBS to the ventromediolateral part of the globus pallidus internus (GPi) with acceptable low complication rates; in order to optimize longterm results in these groups of patient, they will require an interdisciplinary individualized approach both pre- and postoperatively as well as longterm care adjusting to their needs.