Formerly a fatal condition, Wegener's granulomatosis is now treated with good results. Although the annual incidence of Wegener's granulomatosis in the general population is not known, most European and American studies revealed an incidence in the range of one for every 250,000 of the population. The estimated total population of Bahrain as of June 1997 is 586,110; here we report the first diagnosed case of Wegener's granulomatosis in Bahrain. Our patient presented with epistaxis, and from the clinical features, the very high ESR, the operative, histopathological and the radiological findings, and a high index of suspicion we could confirm the diagnosis along with the appropriate immunological test (positive cANCA). Standard treatment with cytotoxic agents and corticosteroids has been used. On follow-up the patient has shown a progressive improvement in her clinical and laboratory parameters.