Background: Frontotemporal dementia (FTD) represents an important group of neurodegenerative diseases, affecting temporal and frontal lobes of both hemispheres. FTD can be divided into three clinical subsyndromes: frontal variant, non-fluent progressive aphasia, and semantic dementia. Abnormalities of the metabolism of Tau protein are present in the physiopathology of FTD, and is found in approximately 50% of sporadic cases, supporting the classification of the FTDs into Tau-negative and Tau-positive subtypes, the latter also called “Tauopathies”. Objective: To review the role of Tau in the pathophysiology of FTD. Methods: Review of the literature on FTD published in the Pubmed and Scielo databases since the year 2000, using the keywords: Tau, Tauopathies, frontotemporal dementia. Relevant references previously published, as indicated in the reference list of selected articles, were also included. Results: Through electronic search we identified 12 articles addressing Tauopathies (ten containing original data and seven reviews), and 20 articles (16 with original data and four reviews) on FTDs. Conclusions: There is consistent evidence in the literature to support the notion that Tau protein plays a crucial role in the pathogenesis of FTDs and other neurodegenerative dementias, and the knowledge on these mechanisms is necessary for the development of more specific therapies.