Frontotemporal dementia is a clinicopathological syndrome caused by progressive degeneration of the frontal lobes, anterior temporal lobes or both. A wide spectrum of cognitive, psychological and behavioural symptoms has been reported, with early disturbance of personality and social conduct, impairment of executive functions and language, while memory and visuo-spatial skills are usually preserved. Published criteria can lead to a good accuracy in the antemortem diagnosis of frontotemporal dementia with a sensitivity of 85% and a specificity of 99%. Treatment rests on the management of behavioural disturbances.