Wegener granulomatosis is characterized by focal necrotizing granulomatosis of the upper respiratory and pulmonary tracts, by a necrotizing vasculitis and focal necrotizing glomerulonephritis. Clinical symptoms typically include intractable sinusitis or persistent nasal obstruction, serous otitis media, hemoptysis and pleurisy. These symptoms can also be accompanied by intermittent fever, weight loss, myalgia and sensory neuropathy. The oral lesions, including palatal ulceration, lingual ulceration, aphthae, nonhealing extraction sockets, gingivitis, have been infrequently described. Wegener's granulomatosis occasionally presents in the early stages as a characteristic hyperplastic gingivitis, named by the authors "strawberry gums", which fails to respond to conventional periodontal therapy. A case is reported, in which this clinically distinctive gingivitis was the presenting lesion with the serous otitis, illustrating that less frequently occurring entities, such as Wegener's granulomatosis, should be considered in the differential diagnosis of localized gingival lesions which fail to respond to conventional therapy.