Objective: To evaluate the treatment and outcomes of malignant neoplasms involving the external and middle ear. Methods: Twelve cases of external and middle ear carcinoma treated from 2014 March to 2020 December were reviewed retrospectively for their clinical characteristics and treatment outcomes, including six squamous cell carcinoma patients and six adenoid cystic carcinoma patients. Ten patients were treated originally at our hospital, including one patient in T1 stage, three in T3 stage and six in T4 stage, according to the University of Pittsburgh-modified TNM staging system. All patients underwent surgery，with eight patients received postoperative radiotherapy and/or chemotherapy. Surgical approach included lateral temporal bone resection (n = 2), subtotal temporal bone resection (n = 9), and lateral skull base tumor resection with infratemporal fossa type B approach (n = 1). Outcomes: The 3-year overall survival (OS) rate was 73%. The positive margin (p = 0.033) was associated with worse OS. Conclusion: The incidence of carcinoma originating from the external and middle ear is low with insidious onset. Surgical resection is the preferred treatment, in combination with postoperative radiotherapy and/or chemotherapy may be an effective comprehensive treatment strategy. Given the rarity of the pathology, many controversies still exist concerning optimal management.