The most life-threatening of the types of EDS is vascular EDS (vEDS); all others are “quality of life” threatening. All forms of EDS are TREATABLE. We have devoted an entire chapter to vEDS due to its high mortality risk, poor prognosis, its influences on life expectancy, and its catastrophe complications, including aneurysms and ruptures. These necessitate specialized interventions and prophylactic treatments. Even though it is a very rare disorder and form of EDS, vEDS should be assessed separately from the group of the other EDS/hypermobile subtypes. vEDS is approximated to contribute up to 5% of EDS cases. However, with more recognition of hEDS, this is probably less. The clinical features of vEDS are from the abnormal COL3A1 gene situated on the long arm of chromosome 2, including four criteria: 1) acrogeria, 2) thin, translucent skin that is pale, smooth, velvety, and soft, with visible vessels, 3) hematomas and ecchymosis, and 4) a history of arterial, obstetrical, or digestive complications. Heartburn is the most commonly reported GI symptom. vEDS involved blood vessel fragility. Even minor trauma, which would not cause a problem, may cause vessel or arterial rupture, and vessels may rupture spontaneously with no history of trauma. Clinical awareness and timely diagnosis of vEDS are still inadequate, and it is usually diagnosed after life-threatening complications or death.