Frontotemporal dementia (FTD) is the second most common type of presenile dementia and the forth most common type of senile dementia, but probably the most costly due to its florid symptom characteristics. Clinically, it often presents with changes of personality, restlessness, disinhibition, and impulsiveness and the clinical features can be complicated by neurological signs, such as motor neuron signs, parkinsonism, and gait disturbances. Syndromatically, FTD can be subdivided into a group with predominating behavioural disturbances (frontal variant) and another with predominating language deterioration (temporal variant). Based on the underlying pathological changes, FTD is nosologically divided into disorders such as Pick's disease, frontotemporal lobar degeneration, corticobasal degeneration, progressive supranuclear palsy, and frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17). The cause in sporadic FTD is most often unknown, but in FTDP-17, one of the hereditary FTDs, there is a causative mutation in the tau gene. The frequency of tau-gene mutations is low in sporadic FTD and present in about 10-40% of hereditary FTD. Other types of hereditary FTD have been described, such as FTD caused by mutations in chromosome 3, chromosome 9, and a FTD syndrome can also be caused by mutations in the presenilin-1 gene. Since there is no curative, treatment of prevailing symptoms is the given alternative. Serotonergic acting drugs have been shown to alleviate behavioural symptoms.