Wegener granulomatosis is an immune-mediated, systemic vasculitis with unknown etiology that can be seen in almost any anatomical site. Positivity for antineutrophil cytoplasmic antigene, which is a serological marker, and presence of granulomatous vasculitis in histopathologic specimens from the lesions are accepted as diagnostic. A case of Wegener granulomatosis whose presenting symptoms and signs are related to otologic and meningeal involvement of the disease is reported. Apart from atypical presentation, histopathological and serological findings from this patient remained negative throughout the course of the disease, and the diagnosis was made on the basis of clinical and secondary laboratory findings. We herewith discuss this unusual case in the light of relevant literature. (c) 2008 Published by Elsevier Inc.