Background: Choriocarcinoma is the most aggressive kind of gestational trophoblastic neoplasms. Although the risk of brain metastasis in GTN is rare, in patients with choriocarcinoma the incidence of brain metastasis is 11%. We have reported a case of choriocarcinoma with brain metastasis which was successfully treated with EMACO regimen. Case presentation: A 34-year-old woman was presented with vaginal bleeding, dyspnea and moderate abdominal pain. She had menstrual delay of about two weeks. She had a primary β-HCG of 132,600 mIU/ml. in lung CT scan, a metastatic lesion with a size of 68×50 mm was observed in the lower lobe of the left lung. The patient underwent D&C which revealed choriocarcinoma. Brain MRI also showed a small metastatic mass with a size of 7 mm at right occipital lobe. The patient was started on chemotherapy with EMACO regimen. The patient’s β-HCG decreased continuously and it was negative after 4th cycle and 6 sessions of radiotherapy. It also remained negative 6 months after chemotherapy. The final examinations of the patients had no abnormal findings. Conclusion: Brain metastasis may be relatively asymptomatic in patients with choriocarcinoma and it should be considered by physicians even when there are no neurological symptoms. Also, EMACO regimen seems to be an appropriate regimen for treatment of metastatic choriocarcinoma.