Abstract: Objective: To discuss the clinical diagnosis, therapies and prognosis of adenoid cystic carcinoma of external auditory canal.Method: Eleven patients with adenoid cystic carcinoma of external auditory canal, who had been admitted to West China hospital, Sichuan university from April 2009 to October 2015 were recruited and a retrospective analysis was performed.Result: All the 11 patients were treated with surgery except one elderly with T1 stage who was only treated with radiotherapy(RT), 1 of the 10 patients treated with surgery were added on radiotherapy after surgery. Following up for 12-104 months, the patient who was treated with RT alone has survived with carcinoma for 51 months by now, among the remainder stage T1 patients, one was reoperated for local recurrence 95 months after surgery, and therefore survived without recurrence. The other 2 patients all survived with free-disease; in the stage T2 patients, one was lost to follow up after identified local recurrence with pulmonary masses 12 months after surgery, another one committed suicide because of family problems 56 months after operation, and the other one survived without recurrence for 57 months after surgery; the stage T3 patient survived without recurrence for 26 months at present; in the T4 patients, one died after gamma knife therapy for local recurrent disease with brain metastasis 22 months after surgery, another one died from pulmonary metastasis 17 months after surgery, and the other one survived without recurrence for 56 months.Conclusion: Adenoid cystic carcinoma of external auditory canal is a rare disease with high misdiagnosis rate and progresses slowly. Surgery is the preferred therapy. The prognosis is closely related to disease clinical stage at presentation type of pathology and surgical approaches. Adenoid cystic carcinoma of external auditory canal is characteristically susceptible to local recurrence and distant metastasis, inaddition to routine re-exam of ear, the patients should be reviewed carefully to exclude of pulmonary, brain and kidney metastasis during follow-up.