Dopa responsive dystonia is a treatable genetic disorder among all dystonias. It is imperative to make a diagnosis of DRD in a child or adolescent presenting with gait difficulty. Therapeutic benefit to levodopa is the first step to diagnosis. Genetic testing is the next step, but studies on molecular biology have shown various sites with different mutations. Standard text book on neurology and paediatrics provide very little description of this disorder. The authors have thoroughly reviewed this disorder and hopefully will be beneficial to all readers and this chapter may act as a source of reference.