Childhood dystonias represent a heterogeneous group of mostly inherited disorders. In this age group, generalized dystonias predominate and cause significant disability. A functional disturbance in the striatal control of the internal globus pallidus causing altered thalamic control of cortical motor areas seems to represent the pathophysiological background. We discuss the etiologic differentiation of primary dystonias from acquired/exogenous causes and heredodegenerative disorders. With the exception of dopa-responsive dystonia, treatment of dystonia is difficult. Besides medical therapy, neurosurgical procedures like bilateral pallidal stimulation show significant clinical benefit in special etiologic groups of dystonia, i.e., primary generalized dystonia.