A case of adenoid cystic carcinoma of the ear in a 67-year-old man is presented along with a review of the literature. Adenoid cystic carcinoma most commonly arises in the salivary glands. Primary cutaneous adenoid cystic carcinoma occurs principally in the scalp and chest. Perineural invasion is seen in at least half the cases and is associated with an increased recurrence rate. Treatment consists of wide local resection with tumor-free margins. Just over half the patients will develop recurrence, with long tumor-free intervals necessitating long-term follow-up. Distant metastases are rarely seen. Radiation therapy is not curative and should be reserved for palliation.