Introduction: We report a case of Ehlers-Donlos syndrome which raised left ventricular pseudo-aneurism at the site of transapical aortic cannulation after acute aortic dissection repair. Case Report: A 49-year-old male underwent total arch replacement for type A aortic dissection using transapical aortic cannulation. CT revealed a pseudoaneurysm at the left ventricular apex 2 weeks after the initial surgery, and the size of pseudoaneurysm increased in another 2 weeks. We performed reoperation and found suture dehiscence of transapical cannulation closure. Post-operative course was uneventful. He was diagnosed as Ehlers-Donlos syndrome by genetic examination after the second surgery. Conclusion: Ehlers-Danlos syndrome might be attributed to vulnerability of cardiac muscle and suture dehiscence, and transapical cannulation should be carefully applied in the patients with Ehlers-Danlos syndrome.