Granulomatosis with polyangiitis (GPA), formerly Wegener's granulomatosis, commonly presents primarily with otitis media and hearing loss, as well as upper and lower respiratory symptoms. However, facial nerve paralysis is a rare manifestation of this uncommon necrotizing vasculitis. We report a patient with facial paralysis accompanied by otitis media. In further studies, our patient was diagnosed with GPA, which was neglected before. In such a presentation, acute suppurative otitis media is the most likely cause of the facial paralysis, but GPA must also be considered, especially in cases with new-onset, painful serous otitis, acute otitis media, or pale granulation tissue in the middle ear, in adults with no previous history of Eustachian tube dysfunction.