Wegener’s granulomatosis is a systemic disease characterized by a granulomatous lesion that can affect any organ throughout the body.

This case-report illustrates the problem posed by a patient presenting with bilateral serous otitis media with marked sensorineural hearing loss, facial nerve paralysis and an atypical serological picture. The importance of early diagnosis and the protocol for the management of a patient with an uncertain diagnosis is discussed. Due to atypical presentations, only a high index of suspicion will ensure an early diagnosis.