Ehlers-Danlos syndrome type 4, the vascular type, is a rare, life-threatening 
inherited disorder of the connective tissue. Affected patients are at risk of 
arterial, bowel and uterine rupture during pregnancy. Generally, this syndrome 
remains undiagnosed until a sudden, acute presentation with organ rupture, and 
results in premature death, even if the patients survive the first and second 
major complications. An early diagnosis with genetic assays can help to plan the 
best treatment, which is often challenging due to the frailty of the arterial 
tissue. We report on a 28-year-old lady who presented with spontaneous rupture 
of a pseudoaneurysm of the posterior tibial artery.