Frontotemporal dementia (FTD) is a common cause of dementia that encompasses 3 clinical subtypes: a behavioral/dysexecutive (frontal) variant and 2 variants with prominent language impairments. There are currently no Food and Drug Administration-approved medications for FTD although symptomatic treatments such as selective serotonin reuptake inhibitors and atypical antipsychotic agents are frequently used to manage behavioral abnormalities associated with this disorder. Evidence for the use of currently available symptomatic treatments in each FTD clinical subtype is reviewed. In addition, the implications of new genetic and neuropathologic information about FTD for the design of future clinical trials and the eventual development of FTD-specific disease-modifying treatments are discussed.