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Pseudoxanchoma elasticum (PXE) is an inherited disorder of connective tissue, characterized by calcification and degeneration of elastin. Clinical manifestations of PXE are protean, with skin, eyes and arteries being most commonly involved. Although often a benign condition, gastrointestinal hemorrhage is a potentially fatal complication. An unusual case of gastrointestinal hemorrhage in a patient with PXE is described and the pathophysiology, clinical presentation and complications of this rare condition are reviewed.