Frontotemporal dementia (FTD) accounts for approximately 10%–20% of all dementias and is the second most common form of early-onset dementia after Alzheimer’s disease (AD). Four distinct clinical subtypes of FTD have been identified: behavioral variant FTD (bvFTD) and three variants of primary progressive aphasia (PPA) syndromes: a semantic variant PPA or semantic dementia (svPPA or SD), a nonfluent variant (PNFA), and logopenic variant (lvPPA). BvFTD is characterized by a progressive deterioration of personality, social behavior, and cognition and is the most common of the FTD subtypes. Executive dysfunction and social cognitive deficits are the main neuropsychological features that characterize bvFTD. Other primary neuropsychological abilities such as visual perception, spatial skills, and some aspects of memory tend to be generally well preserved. Loss of insight or impaired self-awareness is a characteristic feature, evident in over three-quarters of patients with FTD. Detailed neuropsychological assessment is essential for accurate diagnosis of FTD. Developing a robust evidence base for effective neuropsychological interventions is key to support patients with FTD and their families.