Pleomorphic adenoma of the external auditory canal is a rare disease. It is considered to derive from the ceruminous glands. The objective of this study is to familiarize the clinician with the clinical presentation and treatment of this disease. We report the case of a 58-year-old woman. Complete resection should be applied for cases in which magnetic resonanace imaging (MRI) and computerize tomography (CT) examination indicate no erosion in the bone and cartilage tissue. The patient should be seen regularly for recurrence.