Pseudoxanthoma elasticum is a rare inherited connective tissue disorder, which exhibits genetic heterogeneity. It is characterized by elastic tissue degeneration involving many organ systems, with typical cutaneous, ocular, arterial, and gastrointestinal manifestations. Upper gastrointestinal tract hemorrhage occurs in 13% of patients with pseudoxanthoma elasticum and is often resistant to conventional methods of treatment. A case report involving gastric hemorrhage in a patient with pseudoxanthoma elasticum is presented. The characteristics of pseudoxanthoma elasticum are reviewed, and the management of upper gastrointestinal tract hemorrhage in these patients is discussed.