The concept of a disease called ANCA-related vasculitic otitis media (OMAAV: Otitis Media with ANCA Associated Vasculitis) has been proposed as one of the causes of refractory otitis media, and awareness about this disease has been reported to be useful for early diagnosis and treatment of acute otitis media. We diagnosed OMAAV based on histopathological detection of granulomatous lesions and vasculitis lesions in biopsy specimens of the middle ear and inferior turbinate in a patient who had been diagnosed as having acute mastoiditis and been treated by mastoidectomy at another hospital and presented to us with facial nerve paralysis and hypertrophic pachymeningitis. We believe that early intervention will be possible through further improvement of the diagnostic algorithms for intractable otitis media, diagnostic criteria for OMAAV, and promotion of awareness about these disease entities.