Pseudoxanthoma Elasticum (PXE) is a rare inherited elastic tissue disorder with degeneration of elastic fibres involving mainly skin, eyes and the cardiovascular system. Upper gastro-intestinal hemorrhage occurs in 13% of cases and is often resistant to non-surgical methods of treatment. One case report involving recurrent gastric hemorrhage in a young patient with PXE and treated with total gastrectomy is presented. Diagnosis was made, after surgery, by biopsy of skin lesions of the neck. The characteristics of PXE are reviewed and the case is discussed.