Frontotemporal dementia (FTD) is the second most common type of presenile dementia and is the most common form of dementia with the onset before 60 years of age. Its typical symptoms include behavioral disorders, affective symptoms, and language disorders. The FTD is a genetically and pathologically heterogeneous degenerative disorder. Animal models have provided more insights into the pathogenic mechanisms. There are currently no medications that are specifically approved for the treatment of FTD by the Food and Drug Administration. In this article, we review the recent advances in the molecular pathogenesis, pathology, animal models, and therapy for FTD. Better understanding of the pathogenesis and the use of animal models will help develop novel therapeutic strategies and provide new targets for FTD treatment.