Wegener's granulomatosis (WG) is a systemic granulomatous vasculitis that typically affects the upper airways, lungs and kidneys. This form of vasculitis is extremely rare in children, the most common form of paediatric systemic vasculitis being Henoch-Schönlein purpura (HSP). Children with systemic vasculitis look ill and have multiple constitutional complaints, often with prolonged fever. As in adults, WG in children is a multisystem disease. There are no precise statistical data concerning ocular involvement in Wegener's granulomatosis in children. Ocular disease occurs in 50%-60% adult patients with WG and may be an extension of the sinus inflammation or an orbital pseudotumour independent of the sinus disease (15%-50%); sometimes it presents as an ocular myositis. The most common presenting sign of orbital disease is ptosis and anterior displacement of the eye, pain, blurred vision, eyelid swelling and reduction of eye motility. Here we present the case of a 7-year-old girl with WG whose initial manifestation was pseudotumour orbitae. This patient was negative for an initial c-ANCA test. Biopsy specimens obtained from the lacrimal gland and kidney demonstrated granulomatous vasculitis. Treatment with glucocorticoids and cyclophosphamide led to complete, long-lasting remission.