Primary adenoid cystic carcinoma of the external auditory canal constituted a rare group of lesions and is thought originating from ceruminous gland of the external auditory canal. It most often arises in the major and minor salivary glands of head and neck but has been noted in the trachea, breast, skin, middle ear and elsewhere. This tumor is characterized by a very slow growth, high incidence of local recurrence after surgical removal, prolonged history of otalgia and potential for distant metastasis. Recently we experienced a case of adenoid cystic carcinoma from external auditory canal of 26 years old male patient, verified histopathologically. We treated the patient surgically combined with postoperative irradiation, so report it with the literatures.