Vasculitis, defined as an inflammation of blood vessels resulting in ischemic tissue lesions, is a feature of many different diseases and symptoms in childhood. A uniform classification based on characteristic clinical symptoms and histopathological signs is as yet not possible. Most vasculitic syndromes are rarely seen in childhood. This review deals with the more frequently encountered vasculitic syndromes such as polyarteritis nodosa, Kawasaki disease and Henoch-Schönlein purpura. A survey of clinical and histopathological findings as well as recent insights in the pathogenesis and pathophysiology will be given.