[ABSTRACT] INTRODUCTION: Frontotemporal dementia (FTD) is a neurodegenerative disease characterizedby the circumscribed atrophy of the frontal and temporal lobes along with progressive andinsidious changes in behaviour and/or language impairment. OBJECTIVE: To review FTD,emphasizing its genetic bases. Methods: Review of the current medical literature about thesubject. RESULTS: Families with a dominant autosomic inheritance pattern of this diseasehave been recently studied and different