Adenoid cystic carcinoma of the head and neck is relatively rare and is characterized by slow evolution, multiple recurrences, protracted clinical course, and late distant metastasis. This article presents its peculiar clinical course, response to therapy, and long-term treatment results by analyzing 67 cases treated from 1979 to 1991 at the Seoul (Korea) National University Hospital. The most common primary site was the parotid gland in the major salivary glands and the nose and paranasal sinuses in the minor salivary glands. The local control rate was 71.1% at 5 years and 44.3% at 8 years. Late-occurring distant metastases did not allow a plateau in survival curves, even after 8 years. Our study also revealed that surgery combined with postoperative radiotherapy could yield better local control.