OBJECTIVE: To describe a case of Wegener's granulomatosis (WG) this began with sudden bilateral facial paralysis and discusses the otolaryngologic manifestations of GW. CASE REPORT: SAP, female, 31 years old, referred to the clinic with signs sudden bilateral facial paralysis 3 years ago, right hearing loss and nasal discharge. Weight loss of 11 pounds during this period, asthenia and polyarthralgy. On examination, saddle nose, nasal septal perforation with extensive crusting and yellowish discharge, otoscopy right: tympanic membrane retraction important (atelactatic) and normal left, oroscopy normal. Bilateral facial paralysis House-Brackmann IV. Endoscopy: middle meatus and recesses esphenoetmoidal obliterated by edema and crusting. Septal perforation with crusting. Septal mucous with subsequent adhesions. Nasopharynx with synechiae near the eustachian tubes. Audiometry: deafness right and mild left sensorineural hearing loss. CT of the paranasal sinuses: left maxillary sinus with reduced dimensions and destruction of anterior lower portion of the nasal septum. Mastoid CT: opacification of the right mastoid. Chest CT: pulmonary granulomas and latent pulmonary focal point. Laboratory tests: Antibody-neutrophil cytoplasm: positive, anti-proteinase 3 antibody: positive, positive protein C and ESR = 48mm. The patient underwent biopsy of the region of septal perforation and evaluation of Rheumatology and the diagnosis of Wegener's granulomatosis. In use of methotrexate with partial improvement of symptoms, maintaining clinically stable. CONCLUSION: The bilateral facial paralysis by GW is rare but should always be remembered as the early diagnosis results in appropriate specific treatment and, consequently, better quality of life for patients.