Huntington's disease (HD) is a hereditary neurodegenerative disorder that causes chorea and motor dysfunction due to a mutation in the Huntingtin (HTT) gene. Tetrabenazine (TBZ) is used to treat HD-related chorea, but its efficacy and safety require further investigation. This systematic review aims to assess the efficacy and safety of TBZ in reducing chorea and improving motor function in HD patients. A comprehensive search was conducted across multiple sources, including PubMed, PubMed Central, Cochrane Library, Wiley Library, and Google Scholar. Medical subject heading (MeSH) terms were used to enhance search precision. Narrative reviews, clinical practice guidelines, open-label trials, and observational studies were included. Data synthesis followed Cochrane's recommendations for narrative synthesis. Evidence from narrative reviews, clinical guidelines, and trials consistently supports TBZ's efficacy in reducing chorea and improving motor function in HD patients. However, potential side effects like sedation and depression have been noted. This review underscores TBZ's positive impact but emphasizes cautious consideration of associated risks, informing clinical management and further research directions.