Background: Fat embolism (FE) continues to be mentioned as a substantial complication following acute femur fractures. The aim of this systematic review was to test the hypotheses that the incidence of fat embolism syndrome (FES) has decreased since its description and that specific injury patterns predispose to its development. Materials and Methods: Data Sources: MEDLINE, Embase, PubMed, and Cochrane Central Register of Controlled Trials databases were searched for articles from 1 January 1960 to 31 December 2019. Study Selection: Original articles that provide information on the rate of FES, associated femoral injury patterns, and therapeutic and diagnostic recommendations were included. Data Extraction: Two authors independently extracted data using a predesigned form. Statistics: Three different periods were separated based on the diagnostic and treatment changes: Group 1: 1 January 1960–12 December 1979, Group 2: 1 January 1980–1 December 1999, and Group 3: 1 January 2000–31 December 2019, chi-square test, χ2 test for group comparisons of categorical variables, p-value < 0.05. Results: Fifteen articles were included (n = 3095 patients). The incidence of FES decreased over time (Group 1: 7.9%, Group 2: 4.8%, and Group 3: 1.7% (p < 0.001)). FES rate according to injury pattern: unilateral high-energy fractures (2.9%) had a significantly lower FES rate than pathological fractures (3.3%) and bilateral high-energy fractures (4.6%) (p < 0.001). Conclusions: There has been a significant decrease in the incidence of FES over time. The injury pattern impacts the frequency of FES. The diagnostic and therapeutic approach to FES remains highly heterogenic to this day.