Frontotemporal dementia, a heterogeneous neurodegenerative disorder, is a common cause of young onset dementia (i.e. dementia developing in midlife or earlier). The estimated point prevalence is 15-22/100,000, and incidence 2.7-4.1/100,000. Some 25% are late-life onset cases. Population studies show nearly equal distribution by gender, which contrasts with myriad clinical and neuropathology reports. FTD is frequently familial and hereditary; five genetic loci for causal mutations have been identified, all showing 100% penetrance. Non-genetic risk factors are yet to be identified. FTD shows poor life expectancy but with survival comparable to that of Alzheimer's disease. Recent progress includes the formulation of up-to-date diagnostic criteria for the behavioural and language variants, and the development of new and urgently needed instruments for monitoring and staging the illness. There is still need for descriptive population studies to fill gaps in our knowledge about minority groups and developing regions. More pressing, however, is the need for reliable physiological markers for disease. There is a present imperative to develop a translational science to form the conduit for transferring neurobiological discoveries and insights from bench to bedside.