Frontotemporal lobar degeneration （FTLD） is a non-Alzheimer dementia syndrome characterized by focal atrophy of the frontal and/or temporal lobes. Recently, it has been found that FTLD is related to the degeneration of tau protein, and is closely associated with corticobasal ganglionic degeneration, progressive supranuclear palsy, and motor neuron disease. This article reviews the progress in etiology, genetics, pathology, clinical features, and diagnostic criteria of FTLD