The aim was to conduct a systematic review of studies that described the functional profile of patients with behavioral variant frontotemporal dementia (bvFTD), published between 2000 and 2013. The bibliographic search was conducted using the terms "frontotemporal dementia" and "frontotemporal lobar degeneration" in combination with "independence," "functionality," "basic activities of daily living," "disability," and scales that measure functional performance: "Disability Assessment for Dementia-DAD," "Functional Activities Questionnaire (FAQ)," "Direct Assessment of Functional Status (DAFS)." To be included in the review, the study had to mention the characterization of the functional status of patients with bvFTD in the objectives of the study, using a previously validated instrument of functional assessment. Fourteen studies met this criterion. The reviewed studies suggested that individuals with bvFTD have greater functional impairment when compared to those with other subtypes of frontotemporal lobar degeneration or Alzheimer's disease. The studies documented a significant association between cognitive impairment and measures of functionality in these patients. The cognitive profile of patients may predict faster functional decline.