Summary Bilateral temporal bone involvement of eosinophilic granuloma is extremely rare. It has been estimated that approximately 22 cases have been reported so far in the world literature. In many cases, the symptoms are misinterpreted as otitis media, cholesteatoma, or mastoiditis. We report a case of bilateral temporal eosinophilic granuloma with otic capsule involvement, who was initially misdiagnosed and treated for non-specific otitis media. After mastoidectomy and chemotherapy with vinblastine and prednisone the patient's bony lesions of the temporal bone incompletely reossified and labyrinthine function was not satisfactory. Children with otitis media or mastoiditis with no response to medical treatment should be evaluated with Computerized tomography (CT) scanning of the temporal bone.