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We retrospectively reviewed 35 cases of adenoid cystic carcinoma that had originated in the minor salivary glands of the paranasal sinuses or nasal cavity. All patients had been seen at two tertiary-care referral centers in western New York State between 1960 and 2000. Twenty patients had been treated with surgery and adjuvant radiotherapy, 10 patients with surgery alone, three with radiotherapy alone, and two with concurrent radiotherapy and chemotherapy. During the study, 22 patients developed recurrent disease--11 locally; three distantly; seven locally and distantly; and one locally, regionally, and distantly. At the conclusion of the study, 14 patients were alive and disease-free, and eight were alive with disease; 10 patients had died with disease, and three had died of other causes with no evidence of disease. Adenoid cystic carcinoma of the paranasal sinuses or nasal cavity is an aggressive neoplasm that results in a high incidence of both local recurrence and distant metastasis, regardless of treatment modality. Most cases are ultimately fatal, although long disease-free intervals have been observed. A combination of surgery and radiotherapy offers these patients the best chance for disease control.