Dystonia may be a sign or symptom, that is comprised of complex abnormal and dynamic movements of different etiologies. A specific cause is identified in approximately 28% of patients, which only occasionally results in specific treatment. In most cases, treatment is symptomatic and designed to relieve involuntary movements, improve posture and function and reduce associated pain. Therapeutic options are dictated by clinical assessment of the topography of dystonia, severity of abnormal movements, functional impairment and progression of disease and consists of pharmacological, surgical and supportive approaches. Several advances have been made in treatment with newer medications, availability of different forms of botulinum toxin and globus pallidus deep brain stimulation (DBS). For patients with childhood-onset dystonia, the majority of whom later develop generalized dystonia, oral medication is the mainstay of therapy. Recently, DBS has emerged as an effective alternative therapy. Botulinum toxin is usually the treatment of choice for those with adult-onset primary dystonia in which dystonia usually remains focal. In patients with secondary dystonia, treatment is challenging and efficacy is typically incomplete and partially limited by side effects. Despite these treatment options, many patients with dystonia experience only partial benefit and continue to suffer significant disability. Therefore, more research is needed to better understand the underlying cause and pathophysiology of dystonia and to explore newer medications and surgical techniques for its treatment.