Ehlers-Danlos syndrome (EDS) is a hereditary connective tissue disorder that currently includes seven known subtypes. The clinical picture is characterized by varying degrees of skin hyperelasticity, hypermobile joints, and fragility of blood vessels and tissues. The very rare and complex nature of the disease necessitates that physicians in practice and clinics can quickly yet accurately gain an overview of diagnostics and therapy. The matrix of the connective tissue is disrupted in EDS. Most of the known EDS types are caused by mutations in genes that affect the collagen structure or enzymes involved in collagen metabolism. The prevalence is estimated to be around 1 in 5,000 to 10,000 births. The most common and well-known form, referred to as the classical type of EDS, is based on a majority of cases.