A 16-year-old African-American girl presented to our institution with a 6-week history of swollen ankles and 5-week history of conjunctival injection. She had previously been treated with antibiotics for suspected lower extremity cellulitis. Bilateral ankle swelling with erythema and episcleritis were remarkable clinical findings. Laboratory investigations showed: normal CBC and differential, ESR 68 mm/hr, serum BUN 15 mg/dL, and serum creatinine 1.0 mg/dL. Urinalysis showed proteinuria and hematuria although 24-hour urine collection was normal for protein. Antinuclear antibody (ANA) and anti-proteinase-3 antibodies (C-ANCA) were detected while anti-myeloperoxidase antibody (P-ANCA) was absent. CT scan of the chest showed multiple granulomas in both lungs. MRI of the paranasal sinuses, orbits and brain were unremarkable. With a diagnosis of Wegener's granulomatosis (WG), she was started on intravenous solumedrol and cyclophosphamide and maintained on daily oral doses of both medications. Two weeks after initiation of therapy, she was admitted with acute renal failure and massive edema of the labia majora and pitting edema of lower extremities. WG is a granulomatous vasculitis affecting the respiratory tract and kidneys. It occurs mostly between the ages of 20 and 40 years and appears to affect men and women equally. While glomerulonephritis is seen in about 25% of patients at the time of diagnosis, mucosal inflammation of the respiratory tract is reported in as many as 75% of cases. The patient presented here is an unusual case of WG occurring in adolescence with involvement of the lower respiratory tract and rapid progression to acute renal failure.