Nasopharyngeal adenoid cystic carcinoma is a rare tumor. Compared with other nasopharyngeal tumors, it is characterized by slow evolution but is locally aggressive and has a high tendency to recurrence. Due to the rarity of cases, no consensus exists about treatment approaches. We report the case of 42-year-old man with a locally advanced adenoid cystic carcinoma. The patient received concurrent chemoradiation and had a good objective response. After one year, he developed lung metastasis and received platinbased chemotherapy. The aim of this work is to review the literature concerning this rare malignancy and discusses treatment approaches, both for initial management and locoregional/metastatic relapses.