Since the discovery of EDS, five different classification systems have been used by clinicians. The Villefranche nosology, the most recent, recognized six EDS subtypes according to major and minor clinical criteria. Since the introduction of the Villefranche nosology, research in EDS has expanded, and new subtypes were discovered. An updated classification system for EDS was proposed by the International Consortium on EDS, which defined 13 clinical subtypes of EDS according to the clinical manifestations. Major and minor clinical criteria were proposed for each subtype. Each subtype was given a name that describes a characteristic phenotype manifestation. On March 15, 2017, the American Journal of Medical Genetics published the internationally agreed-upon 2017 EDS Classifications for the first time since 1997. The 13 subtypes of EDS, according to the 2017 International Classification, are listed, listing underlying genetic and pathogenetic mechanisms and the major and minor diagnostic criteria.