Granulomatosis with polyangiitis (GPA), also known as Wegener's granulomatosis, is a rare disease in childhood. Of 39 GPA patients that we diagnosed during a 20-year period, only 3 (7.7%) were younger than 18 years. We report the course of GPA in three girls whose disease started at the ages of 16, 11, and 6 years. All had cutaneous manifestations: the first had necrotizing vasculitis, the second had palpable purpura, and the third had right upper-eyelid edema and infiltration and proptosis caused by extraocular pseudotumor, initially histologically misdiagnosed as orbital immunoglobulin G4 (IgG4)-related disease. Unlike with skin vasculitis and glomerulonephritis, upper-airway and orbital inflammation were resistant to immunosuppressive therapy. Our report emphasizes that children presenting with cutaneous vasculitis, chronic eyelid swelling, sinusitis, or hoarseness should be tested for antineutrophil cytoplasmic antibodies. We emphasize that the upper-eyelid edema and infiltration, with histologic characteristics of orbital IgG4-related disease, may be the initial presentation of localized GPA in children, a feature that, until now, has been described only in adults.