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Peripheral arterial involvement is common in pseudoxanthoma elasticum. It can be the first symptom. Small and medium size arteries are most often involved. The arterial lesions are diffuse, stenotic and often with marked calcium deposition. This arterial involvement can be developed early and if happening in young patients it is of great diagnostic significance. Peripheral vascular disease is most common in the limbs. Its evolution is remarkably slow. Coronary arterial disease can induce angina pectoris but seldom myocardial infarction. In cerebral arteries, lesions consist in stenoses and aneurysmal dilatation. They are responsible for thrombotic or hemorrhagic accidents. High blood pressure is frequent. It increases the hemorrhagic risk. Aortic lesions are exceptional, and if encountered one must discuss a Marfan disease association. Visceral hemorrhages, gastrointestinal or uterine, often the first symptom, are secondary to arteriolar lesions. Histologic studies reveal narrowed arterial lumen, due to medial thickening. Media is invaded by irregular and anarchic elastic fibers. Elastic laminae are fragmented and disorganized. The abnormal elastic tissue tends to be calcified. When compared to the arterial lesions, cardiac involvement is uncommon. It has been described in the first case of literature. It essentially consists in an endocardial thickening of the auricles and of the mitral and tricuspid valves.