[Diagnostic difficulties and unfavorable course Wegener's granulomatosis in 1-year-old girl]

We described an 11 year old girl who presented and unusual clinical course of Wegener's granulomatosis (WG). The rapidly progressive glomerulonephritis was the onset of the illness. The characteristic manifestation of WG from the respiratory tract was observed after 1.5 year from the beginning of the disease. Inspite of intensive treatment the end-stage renal failure developed and progress of the disease was observed. These complications caused patient's death.