A 65 year old woman with aortic insufficiency, joint hypermotility, platypody, arachnodactyly and ptosis of visceral organs is described. This case did not show hyperelastic skin, ecchymoses, atrophic lesions, molluscoïd pseudotumors or eye changes. There was however a periocular hyperelastic skin. Skin biopsy revealed that the elastic fibers were abnormally numerous and fractured in some parts. Collagen fibers were normal. The patient had also diabetes mellitus and chronic-pyelonephritis with relapsing macroscopic hematuria. Patient’s son also shows joint hypermotility and flat feet. One of her two daughters presents joint hypermotility. Our case apparently constitutes an example of incomplete degenerative and hereditary disorder of the connective tissue, related to the Ehlers-Danlos syndrome. In every case of aortic insufficiency with obscure aetiology, somatic examination and family investigation are strongly indicated.