This study aimed to review the scientific literature on etiopathogenic factors of savant syndrome associated with autism spectrum disorder (ASD). Searches were performed in the following databases: Medical Literature Analysis and Retrieval System Online, Embase, Scopus, Science Direct, Cochrane Library, Latin America and Caribbean Center on Health Sciences Information, and Google Scholar, as well as gray literature and indirect search. A total of 42 studies were included in the present review, and the etiopathogenic theories of each study were assessed. Despite more than 100 years of reports on savant syndrome, most studies on this topic still comprise subjective opinions or qualitative analysis. In the last 3 decades, we found more structured studies with relevant methodology. There are still large gaps in knowledge regarding the concept, etiopathogenic theories, and psychometric assessment of savant syndrome associated with ASD, which indicates the need for future research.