Much of our current understanding of the pathogenic mechanisms in human neurodegenerative disorders has been derived from animal studies. As such, transgenic mouse models have significantly contributed to the development of novel pathogenic concepts underlying human tauopathies, a group of diseases comprising various forms of neurodegenerative disorders including Alzheimer's disease, corticobasal degeneration, argyrophilic grain disease, progressive supranuclear palsy, and Pick's disease as well as hereditary fronto-temporal dementia with parkinsonism linked to chromosome 17. Here, we will review in vivo models of human tauopathies with particular preference to transgenic mouse models. Strengths and limitations of these models in recapitulating the complex pathogenesis of tauopathies will be discussed.