Ehlers-Danlos syndrome denotes a group of inherited connective tissue diseases 
comprising nine types. Type IV Ehlers-Danlos syndrome is the most 
life-threatening form. It is characterized by a type III collagen deficiency 
resulting in arterial fragility and death from vascular rupture or bowel 
perforation. This disease involves a col 3A1 gene mutation. We report the case 
of a 44 year-old woman with type IV Ehlers-Danlos syndrome. The medical history 
of our patient included bowel necrosis and two vascular ruptures. We indicate 
data required to establish Ehlers-Danlos syndrome diagnosis and guidelines for 
patient management.