Tetrabenazine works mainly as a VMAT-inhibitor and as such promotes the early metabolic degradation of monoamines, in particular the neurotransmitter dopamine. Tetrabenazine (TBZ), a catecholamine-depleting agent initially developed for the treatment of schizophrenia, when tested for other indications, has proven to be more useful for the treatment of a variety of hyperkinetic movement disorders. These disorders include neurological diseases characterized by abnormal involuntary movements such as chorea associated with Huntington’s disease, tics in Tourette’s syndrome, dyskinesias and dystonias in tardive dyskinesia, also primary dystonias and myoclonus. It will also review the chemistry, pharmacokinetics and dynamics of the drug and its mechanism of action compared to that of reserpine, the only similar compound.