An 85-year-old woman presented with rapidly progressive hearing loss, dyspnea, and multiple nodular shadows on her chest X-ray. On examination, she had bilateral exudative otitis media, sore throat, hemoptysis, fever, and polyarthralgia. Bronchoscopic examinations showed edematous airway mucosa, and transbronchial lung biopsy revealed necrotic granulomatous inflammation surrounded by epitheloid cells with multinucleated giant cells. The diagnosis of limited type of granulomatosis with polyangitis (Wegener's granulomatosis) was confirmed despite the absence of ANCA. She developed facial paralysis just before the diagnosis. Immunosuppressive therapy with cyclophosphamide, prednisolone, and sulfamethoxazole-trimethoprim was initiated. All of the above-described clinical symptoms improved rapidly. Facial nerve paralysis in association with granulomatosis with polyangitis is rare, but clinicians should consider this disease in the list of differential diagnoses even in the case of ANCA negativity.