Huntington's disease (HD) is an autosomal dominant, inherited, neuropsychiatric disease that gives rise to progressive motor, cognitive and behavioural symptoms. Current drug therapy has no effect on the progression of disability, and the need for any pharmacological treatment should be carefully considered. Hyperkinesias and psychiatric symptoms may respond well to pharmacotherapy, but neuropsychological deficits and dementia remain untreatable. Pharmacological intervention in the treatment of the movement disorder of HD is aimed at restoring the balance of neurotransmitters in the basal ganglia. A surprising amount of current drug therapy of HD in clinical practice is based on studies published before 1990. The authors conducted a systematic review of pharmacological therapy in HD using the available papers that were published between 1990 and 2006.