In this paper, we reconstructed the medical history of frontotemporal dementia (FTD) by reviewing the literature and analyzing papers with the highest impact through citation index. Several research studies and groups involved in FTD have been reviewed. An increasing amount of knowledge has been made available in the last 20 years through a large number of publications, leading to a better definition of the genetic and clinical bases of the disease. A total of 1,436 references (articles and reviews), published in 395 journals, were retrieved through the Scopus database. The two highest publication peaks (i.e., largest number of publications) were found in 2000 and 2008. The most cited papers considering both total citation number and the number of citations within the first two years after publication refer to: (i) the genetic bases of FTD, (ii) the clinical criteria that progressively refined the different FTD phenotypes, and (iii) FTD epidemiology. Advanced neuroimaging techniques, genotype-phenotype heterogeneity, and animal models gave us a broader understanding of various aspects of the disorder. These findings confirm the great interest in FTD research. The analysis of the literature might help in guiding future goals in the field.