In Western population, sinonasal malignant lymphoma is rare and constitutes 1.5% of all non-Hodgkin lymphoma (NHL) and 2.2% of extranodal lymphomas. Wegener's granulomatosis (WG) is the necrotizing vasculitis of small arteries and veins. WG is characterized by granulomatous vasculitis and involves the upper and lower respiratory tract together with glomerulonephritis. But there are some forms of WG named limited WG that involves the upper respiratory tract only without glomerulonephritis and even seronegative without renal involvement. Herein, we present a typical WG with isolated sinonasal tract involvement with clinical, and radiological findings with the final diagnosis of NK/T-cell angiocentric lymphoma by the repeated biopsies. Since both diseases have same clinical and radiological findings differential diagnosis may be difficult.