Wegener's granulomatosis (WG) is a multi-system disease characterized by granuloma formation and widespread necrotizing vasculitis. It classically presents with involvement of the upper and lower respiratory tracts and renal system. Facial nerve palsy and otologic manifestations have been reported during the course of the disease but it is extremely rare as the presenting features. In Literature only one case of bilateral facial palsy as presenting sign of WG is reported. The testing of anticytoplasmic antibodies versus neutrophil polymorphonucleate granules (c-ANCA) are highly specific for the diagnosis of WG, being positive in 97% of the cases. The early diagnosis and the timely medical treatment lead to high rates of remission of an otherwise lethal disease. A case of WG presenting with bilateral facial paralysis and bilateral serous otitis media is reported.