Wegener's Granulomatosis (WG) is an immunologically mediated rare multisystem disease characterized by necrotizing granulomatous inflammation affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. WG oral lesions are reported to occur in 6%-13% of patients; they were an initial presenting feature in only 2% of cases. A case of strawberry gingivitis as the first sign of WG presented with erythematous and painful localized gingival enlargement affecting the maxillary anterior region of jaw in a 17-year-old boy is reported here. Correlation of histopathology with routine hematoxylin and eosin and special stains such as Grocott-Gomori's methenamine-silver nitrate and periodic acid-Schiff, peripheral blood smear, chest radiographs, Mantoux test and clinical presentation were established in diagnosing this rare entity. By the above-mentioned procedures, we arrived at the diagnosis of WG. The aim of reporting this case was to emphasize that, the dental surgeon often being the first person to examine the oral cavity, should be familiar with the typical appearance of gingiva as "strawberry gingivitis," its clinical course, diagnostic parameters and adequate management of WG.