The case is reported of a 69-year-old female with atrophic papules on the skin who developed multiple spontaneous intestinal perforations of which she eventually died. The skin lesions in combination with lesions in the gastrointestinal tract are typical for Degos' disease or malignant atrophic papulosis. The characteristic histopathological and endoscopic features of this rare disease are reported. This case demonstrates the importance of routinely performing endoscopy in Degos' disease to detect silent perforation, even in patients without gastrointestinal complaints.