Wegener's granulomatosis (WG), characterized by a necrotizing granulomatous vasculitis, is a rare systemic disease particularly infrequent in children. We report an unusual case of WG in a 16-year-old male patient in whom the cutaneous manifestations were the presenting signs that preceded the upper respiratory symptoms by several months. In addition, the finding of a calcified pulmonary lesion has never been reported in association with WG. Accurate diagnosis of early skin lesions is important in view of the available effective therapy. Clinical and pathologic features of WG and treatment are reviewed.