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Granulomatosis with polyangiitis (GPA) is considered as a systemic triad of necrotizing granulomatous vasculitis of the upper and lower respiratory tracts (pulmonary vasculitis) and a rapidly progressive glomerulonephritis. Few cases of a superficial form with only oral involvement are reported. The purpose of this article is to describe the steps in reaching a conclusive diagnosis in lesions involving the palate. Since in most cases the histopathological report will turn inconclusive, proper clinical evaluation and definitive diagnostic test like the estimation of cytoplasmic-antineutrophilic cytoplasmic antibody will be mandatory for obtaining a conclusive result.