Huntington's disease (HD) is a heredodegenerative neurological disorder with chorea and other hyperkinetic movement disorders being part of the disease spectrum. These along with cognitive and neurobehavioral manifestations contribute significantly to patient's disability. Several classes of drugs have been used to treat the various symptoms of HD. These include typical and atypical neuroleptics along with dopamine depletors for treatment of chorea and antidepressants, GABA agonists, antiepileptic medications, cholinesterase inhibitors, antiglutamatergic drugs and botulinum toxin for treatment of other manifestations. Tetrabenazine (TBZ), a dopamine depleting medication was recently approved by the US FDA for treatment of chorea in HD. The purpose of this article is to briefly review information regarding HD and current treatments for chorea and specifically focus on TBZ and review the literature related to its use in HD chorea.