In this study, we have identified a cohort of children with hypermanganesemia and progressive parkinsonism–dystonia who carry homozygous loss-of-function mutations in SLC39A14. We show that disodium calcium edetate (Na2CaEDTA) effectively chelates Mn and increases urinary Mn excretion in affected individuals. The gene discussed is SLC39A14; the disease is Dystonia.