The clinical and histological diagnosis of Degos' syndrome could be made in a 8-month-old infant with scarring skin lesions. Some weeks later the baby developed severe intestinal symptoms and died from perforations of multiple necrotic lesions of the small bowel. Autopsy revealed in addition to the skin and gut lesions a haemorrhagic cortical infarction of the brain and fibrotic areas in the myocardium. In all these lesions characteristic stenotic endovascular proliferations were seen. Malignant atrophic papulosis is an extremely rare disease in infancy (our patient is the fifth reported case) with a well defined clinical picture, characteristic histological appearance of obliterating vasculitis, but unknown aetiology.