Granulomatosis with polyangiitis (GPA) is a rare autoimmune vasculitis typically affecting the respiratory tract and kidneys. This case report describes a 33-year-old Saudi female presenting with persistent otitis media, progressive hearing loss, and facial nerve paralysis unresponsive to conventional treatment. She later developed systemic symptoms, including oral ulcers, gastrointestinal bleeding, and a cecal mass. Histopathological analysis of the middle ear and cecal tissue confirmed GPA, despite the absence of renal or pulmonary involvement. Serology revealed elevated PR3 antibodies. The patient showed marked improvement following immunosuppressive therapy with steroids and rituximab. This case underscores the need to consider GPA in patients with atypical, treatment-resistant otologic symptoms and highlights the importance of early recognition and multidisciplinary management to prevent irreversible complications.