We describe a woman who presented with syncopal episodes and unilateral hypoglossal paresis in association with a highly infiltrative retropharyngeal mass. After an extensive malignancy workup, the patient was found to have Wegener granulomatosis (WG), an autoimmune necrotizing vasculitis that presents with inflammatory lesions anywhere in the respiratory tract and variable renal involvement. The archetypal presentation in the head and neck is erosive sinonasal crusting, though otologic, pharyngeal, and laryngeal findings are common. Highly uncharacteristic lesions are occasionally encountered and may contribute to significant diagnostic dilemmas. Neurologic involvement is not uncommon, but few reports of hypoglossal paresis and no reports of syncope as a result of WG are found in a review of the literature. Given the variability of presentation of WG in the head and neck, the otolaryngologist must maintain a high degree of suspicion for this disease in the evaluation of airway lesions.