Huntington's disease (HD) is a severely disabling autosomal dominant neurodegenerative disease. Clinically, it is characterized by a triad of progressively worsening chorea, cognitive decline, and psychiatric disturbances. The pathogenesis of HD is well-established. The disease is caused by CAG trinucleotide repeat expansion in exon 1 of the huntingtin (HTT) gene on human chro- mosome 4. Currently, there are no disease-modifying treatments available for HD, The HD patients mainly receive the symptomatic and comprehensive treatment to improve their symptoms and quality of life. This article provides a comprehensive overview of the current standardized approaches to diagnosis and management of HD. It also summarizes the research progress of molecular therapy based on the pathological mechanism of HD in recent years.