Middle ear adenoma is a rare disease derived from the middle ear mucosa. It is usually lack specific findings and easily mistaken for other conditions, delaying a correct diagnosis. In particular, few cases with facial nerve paralysis have been reported. We describe a case of middle ear adenoma that caused hearing loss and recurrent facial paralysis in a 29-year-old woman. In an attempt to treat the facial nerve paralysis, we performed tympanomastoidectomy and facial nerve decompression. By pathological examination, we finally diagnosed it middle ear adenoma with neuroendocrine differentiation. Retrospectively, if we meet the antibiotic resistant mass in the middle ear, we should suspect the tumor. One year after surgery, the adenoma has not recurred but long-term observation is required.