Ehlers-Danlos syndrome (EDS) is a group of hereditary connective tissue disorders which are clinically characterized by hypermobility of joints, hyperelasticity of skin and tissue insufficiency, sometimes leading to herniation, bleeding, and organ rupture. This is a case report of a 55-year-old man with EDS and a big bladder diverticulum in which we discuss treatment strategy in patients with EDS and bladder diverticula. Initially, treatment approach should be conservative due to high recurrence rates and potential complications following surgical procedures. Invasive procedures - preferably minimally invasive - may be indicated, based on an individual risk evaluation.
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