The morbidity of frontotemporal dementia (FTD), one of the most prevalent dementias praccox, is second to Alzheimer disease (AD). It is different with AD that FTD has a rapider course and a higher mortality. FTD has not yet been fully understood in terms of etiology or pathogenesis, but genetic factors are believed to be involved. In this paper, we were committed to providing a comprehensive overview to FTD in aspects of the neuropathology features and the relevant molecular genetics advances, so that there would be insights to those researchers in search of novel approaches in FTD diagnosis and treatment.