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Background: Brain metastasis of nasopharyngeal carcinoma is very rare and only a few cases has been reported. Typically brain metastasis reveals after diagnosis and treatment of aggressive primary tumor. Diagnosis of metastatic nasopharyngeal carcinoma to the central nervous system at presentation is extremely rare. To the best of our knowledge, our patient is the first-ever reported case of brain metastasis of nasopharyngeal carcinoma at presentation. Case presentation: A 30-year-old woman presented with repeated and severe headache and diplopia at November 2011, and clinical examination revealed increased intracranial pressure. Brain magnetic resonance imaging revealed an enhancing mass at right frontal lobe. A thorough evaluation of whole body revealed no any other problem. Suboptimal resection of the mass by craniotomy revealed metastatic carcinoma. CEA,CK20,CK7 were negative and TTF-1 was positive. Treatment followed by whole brain radiotherapy and chemotherapy with resultant complete improvement of patient symptoms during next 2 years. 17 months after operation, patient noticed left neck adenopathy, but did not followed. Ultimately 8 months later, when she was at 5 months of her first pregnancy, fine needle biopsy of left neck mass revealed undiffentiated carcinoma. It was followed by Panendoscopy" of the head and neck and advanced nasopharyngeal cancer was revealed. Treatment conducted by chemotherapy after 24th week of pregnancy, delivery at term and radiotherapy there after.The patient is alive with no evidence of disease, 15 months after completion of radiotherapy.      Conclusions: Probability of nasopharyngeal origin should be considered at rare instances of brain metastatic carcinoma with unknown origin. Close follow up after treatment of unknown primary brain metastases is mandatory, in order to reveal and control primary site at future. Successful treatment of nasopharyngeal carcinoma at the third trimester of the pregnancy is possible and recommended.