Primary nasopharyngeal adenocarcinoma is uncommon, accounting for less than 0.5% of all nasopharyngeal cancers. As for metastatic nasopharyngeal adenocarcinoma, only sporadic cases have been reported in the literature, mainly from breast, thyroid gland, liver, kidney and lung. We herein describe a 40-year-old male patient, who was diagnosed with a left upper lung adenocarcinoma with brain metastasis. He was referred to our department due to an enhanced signal over the nasopharynx on a follow up PET scan. Biopsy of the lesion proved to be a metastatic tumor from lung cancer. After palliative chemotherapy and radiotherapy to the brain and lung lesions, the nasopharyngeal tumor decreased size remarkably, while the extent of primary lung tumor progressed. The patient finally expired 19 months following the diagnosis of primary lung cancer. Consistent with the findings in literature, the prognosis of such patients is mainly determined by the extent of primary tumor and existence of other distant metastasis. The therapeutic options, however, remains uncertain due to the exceeding rarity of such cases and a lack of enough clinical data. (J Taiwan Otolaryngol Head Neck Surg 2020; 55:208-213)