Pseudoxanthoma elasticum (PXE) is a rare inherited connective tissue disorder which is characterized by elastic tissue degeneration involving the skin, eyes and arteries. We describe the case of a hypercholesterolemic 25 year-old woman with typical genetic dominant form of PXE revealed by distal limb artery involvement after a pregnancy. In young adults with arterial disease, a non atheromatous cause should always be sought for, even if risk factors for atherosclerosis are present.