Objectives. To illustrate the only reported case of Wegener's granulomatosis limited to the head and neck, and to provide an update on current knowledge and treatment of Wegener's granulomatosis. Case report. A 50-year-old man presented with bilateral chronic otitis and mastoiditis, eventually developing bilateral facial palsies. While he progressed with other head and neck symptoms, his disease was limited entirely to the head and neck. Diagnosis of Wegener's granulomatosis was delayed for this reason, due to the lack of systemic manifestations and the inability to confirm the diagnosis with a tissue biopsy, despite an elevated c-ANCA level. Conclusion. The early diagnosis and initiation of appropriate treatment of Wegener's granulomatosis is essential to prevent progression of the disease to a severe and irreversible phase. This case emphasizes that an awareness of this rare presentation is important to prevent the unnecessary delay of appropriate treatment in order to optimize the patient's prognosis, particularly when classic therapy for otitis or facial palsy is ineffective.