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Granulomatosis with polyangiitis (GPA) is a rare disorder characterized by inflammation of small and medium-sized blood vessels (vasculitis) that results in damage to various organ systems of the body, most often the respiratory tract and kidneys. The relapse rate rises from about 20% at 12 months to about 60% at 5 years. The combination of glucocorticoids and cyclophosphamide remains the standard therapy for patients with generalised GPA. Nevertheless, some patients do not respond satisfactorily to this treatment. Here we present a 32 year-old female with relapsing GPA successfully treated with methotrexate for ear, nose and throat (ENT) manifestation.