Wegener's granulomatosis or granulomatosis polyangiitis (GPA) is an uncommon chronic systemic vasculitis in children. The aim of this study was to describe pediatric patients with GPA in Iran. We studied records of all patients with GPA diagnosis who were referred to all Iranian Pediatric Rheumatology Division from 2002 to 2011. A total of 11 patients (5 females and 6 males) enrolled in this study. In children less than 15 years old, the prevalence of GPA is 0.6 per million. The mean age of GPA diagnosis was 11 years and average delay diagnosis was 20 months. Mortality rate was 18.1% due to pulmonary vasculitis and infection. The most common organ system involvement was upper and lower respiratory tract involvement (81.8% and 63.9%, resp.). Other common manifestations were renal (36.3), skin (27.2%), and eye involvement (18.2%).