Summary: Huntington's disease is an autosomal dominant neurodegenerative disorder that is characterized by motor, cognitive, and psychiatric alterations. The mutation responsible for this fatal disease is an abnormally expanded and unstable CAG repeat within the coding region of the gene encoding huntingtin. Numerous mouse models have been generated that constitute invaluable tools to examine the pathogenesis of the disease and to develop and evaluate novel therapies. Among those models, knock-in mice provide a genetically precise reproduction of the human condition. The slow progression and early development of behavioral, pathological, cellular, and molecular abnormalities in knock-in mice make these animals valuable to understand the early pathological events triggered by the mutation. This review describes the different knock-in models generated, the insight gained from them, and their value in the development and testing of prospective treatments of the disease.