Adenoid cystic carcinoma is a common malignancy of the salivary glands. Adenoid cystic carcinoma of the nasopharynx is very rare. The mainstay treatment is surgery with postoperative radiation therapy. We encountered a 57 year-old male with 3- month history of left ear fullness. Otoscopy revealed no abnormal finding and nasopharyngoscopy revealed a mass on left lateral wall of nasopharynx. Biopsy pathology was adenoid cystic carcinoma. MRI showed a 2.75×2.2 cm bulging mass on left lateral wall of nasopharynx with well-defined margin. The tumor was removed successfully by transnasal endoscopic surgery. The patient has been routinely followed up for 11 months, and without local recurrence.