Background: The present study aimed to report the characteristics and treatment outcomes of five patients with nasopharyngeal adenoid cystic carcinoma and a literature review. Methods: Between 2000 and 2009, five consecutive patients (4 men, 1 woman) were diagnosed with nasopharyngeal adenoid cystic carcinoma and treated at our institution. Three patients had stage IVa (T4N0M0) and two patients had stage III (T3N0M0) cancer. Primary treatment consisted of concurrent chemoradiation in three patients andradiotherapy alone in two patients. Surgery was limited to endoscopic biopsy for histological diagnosis. Results: Four patients achieved complete response during or after completion of treatment and remained free of disease for a median of 27 months. Four patients developed local recurrence 8-30 months after initial treatment. The fifth patient is alive and free of disease.Conclusion: The findings of the present study and literature review suggest that local failure is a major problem in adenoid cystic carcinoma of the nasopharynx.