Ehlers-Danlos syndrome type IV, also known as Sack-Barabas syndrome, is a dominant autosomal genetic disease that results in a structural anomaly of type III collagen and causes fragility in blood vessels, intestines, lungs, skin, liver, and spleen, etc. There is a high likelihood of arterial rupture and dissection, very often with no trigger factor whatsoever. Spontaneous arterial rupture is the primary cause of mortality among patients who have the syndrome (78.5%). This arterial accident is rare during childhood, but 25% of patients will suffer an initial episode before the age of 20 and 80% before they reach 40. Table 1 lists the diagnostic criteria for Ehlers-Danlos syndrome type IV.1 - 4