The purpose of this article is to describe the clinical and microscopic findings of oral lesions of Wegener's granulomatosis (WG) in a patient who presented with a limited form of the disease. cANCA estimation remains the definitive diagnostic test for WG but we recommend that both the immunofluorescent and ELISA forms of analysis are performed, and care should be exercised in the interpretation of results. Because both serological tests may be negative in a significant proportion of cases, a tissue biopsy is required to help establish the diagnosis. The biopsy needs to be sufficiently deep to include the granulomatous inflammation required for diagnosis, and multiple histological levels on the tissue may be needed to identify vasculitis.