57-year-old woman with a history of ischaemic heart disease, arterial hypertension and after myocardial infarction was admitted to the university hospital because of progressive hearing loss and fever of unknown origin. Shortly before hospitalization she developed cough, hemoptysis and conjunctivitis. On the basis of clinical presentation Wegener's granulomatosis was suspected. To confirm the diagnosis, CT scans of the chest, sinuses and ears were performed and revealed massive lesions especially in tht tympanic cavity, mastoid antrum and cells. Infiltrations were also observed in sinuses, especially maxillary, and typical granulomas were found in the lungs. Moreover, the biopsy taken from the mucous membrane of the nose showed abnormalities typical of Wegener's granulomatosis. Antineutrophil cytoplasmatic antibodies (ANCA) were also examined. It is of interest that c-ANCA (cytoplasmatic) were negative and p-ANCA (perinuclear) were positive which is rare in this disease. The patient was treated with immunosuppressive drugs (intravenous methylprednisolone, oral prednisolone and cyclophosphamide). Following therapy fever, hemoptysis and conjunctivitis subsided, while inflammatory parameters normalized. This case report presents on unusual clinical manifestation of Wegener's granulomatosis with the leading sign of hearing loss.