The aim of our study was to present the treatment outcome for 7- to 9-year-old pediatric patients with disabling monogenic isolated generalized DYT-<i>THAP1</i> and DYT-<i>KMT2B</i> dystonia after bilateral GPi-DBS.<h4>Patients and results</h4>We present three boys aged <10 years; two siblings with disabling generalized DYT-<i>THAP1</i> dystonia and a boy with monogenic-complex DYT-<i>KMT2B</i>. This evidence concerns the gene THAP1 and Dystonia.