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Neuroendocrine neoplasms of the ear pose a diagnostic challenge because clinical symptoms are no specific. Definite diagnosis is made by histopathological analysis with immunohystochemical evalutation. Therapy consists in a complete surgical resection. Controversial terminology of the neoplasm arises from the differentiation of these tumors composed of both endocrine and exocrine glands. Middle ear localization is an extremely rare presentation but less aggressive than gastrointestinal tract or lung localizations which are the most frequen