Rupture of the upper ankle joint ligaments is a common injury. Therapy in patients with Ehlers Danlos Syndrome (EDS) is a challenge due to the autosomal-dominant collagenopathy. Therapeutic recommendations and long term results in treatment of patients with EDS are rare. Therefore uncertainty exists. We present a case of clinical and radiologic long term results after ligament rupture of the upper ankle joint in a patient with EDS. A literature review tries to reveal current therapeutic strategies in patients with EDS.