BackgroundFrontotemporal dementia accounts for up to 20% of cases of dementia in the presenium, yet remains poorly recognised. Diagnostic criteria have been devised to aid clinical diagnosis.AimsTo provide an overview of clinical and pathological characteristics of frontotemporal dementia and its nosological status.MethodsThe review summarises consensus diagnostic criteria for frontotemporal dementia and draws on the authors'clinical experience of 300 frontotemporal dementia cases, and pathological experience of 50 autopsied cases.ResultsFrontotemporal dementia is characterised by pronounced changes in affect and personal and social conduct. Some patients also develop motor neuron disease. Mutations in the tau gene account for some but not all familial cases of frontotemporal dementia.ConclusionsFrontotemporal dementia is a focal form of dementia, which is clinically and pathologically distinct from other dementias. It represents an important model for understanding the functions of the frontotemporal lobes.