Frontotemporal dementia (FTD) is an increasingly recognized cause of dementia. This review discusses the different FTD clinical syndromes and frontotemporal lobar degeneration (FTLD) pathological correlates as well as new genetic and proteomic findings that have added to our understanding of FTLD pathogenesis. Various diagnostic modalities including the use of biomarkers will also be addressed. Finally we will highlight future directions in the FTD field. More research is needed to elucidate the cellular mechanisms of neurodegeneration in FTLD and improve clinical diagnostic capabilities.