Animal models of human diseases are essentially required to investigate the pathophysiological mechanisms of the diseases, and to test potential therapies for the clinics. However, neurodegenerative diseases including Parkinson's disease (PD) are particularly difficult to model or to recapitulate the features because most of them have multifactorial etiologies and chronically progressive symptoms, although Huntington's disease (HD) has an identified etiology such as an excessive expansion of CAG repeats. In this review, PD and HD which were typical neurodegenerative diseases were studied. The animals of PD were roughly classified into a neurotoxic model and a genetic model, and those of HD were divided into excitotoxic, transgenic, knock-in, and knock-out models. Insights obtained from these animal models of neurodegenerative diseases will guide us toward the understanding of the disease mechanisms, the design of new therapeutic strategies, and finally translation into the clinics.