Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculitis usually affecting the upper and lower respiratory tracts and kidneys. Any organ system can be affected by the pathologic process, which remains an etiologic enigma. Limited forms of the disease are recognized in which few extrapulmonary and no renal lesions occur. Cutaneous manifestations occur in 40% to 50% of patients with WG (1). Early diagnosis is imperative because treatment regimens reduce morbidity and mortality in this potentially fatal disease. We report WG in a 10-year-old boy whose upper respiratory tract symptoms began at age 8 years. The case is illustrative of the difficulties that can be encountered in attempting to make this diagnosis. Skin is an easily accessible organ for biopsy, thus an increasing familiarity with the typical biopsy specimen findings within a consistent clinical setting may aid in earlier diagnosis of WG.