Adenoid cystic carcinoma of the nasal septum is extremely rare. We present the case of a 56-year-old male who complained of nasal bleeding and nasal obstruction for 1 month. A mass arising from nasal septum was found by endoscope. The tumor was removed under lateral rhinotomy and histopathologic examination revealed adenoid cystic carcinoma with cribriform pattern. He then had postoperative radiotherapy. No recurrence was noticed after 1 year of follow-up. Despite its rarity, adenoid cystic carcinoma should be taken into consideration in the differential diagnosis of nasal tumor.