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We report a case of esthesioneuroblastoma with intracranial extension treated with surgical resection and chemotherapy. A 5-year-old child presented with visual disturbance, and was subsequently found to have a huge sized mass in the anterior skull base, suprasellar region and lateral ventricle. A bifrontal craniotomy with excision of the tumor through subfrontal and interhemispheric approach was performed. The patient received postoperative chemotherapy according to Tandem protocol. A follow-up magnetic resonance(MR) image showed that the tumor showed partial regression, however, there was no change in tumor size in lateral ventricle. Second operation with excision of remnant mass through a transcallosal approach was performed. MR image, 9 months after first operation showed the postoperative change in frontal area without enhancing mass. By employing staged operation and chemotherapy, the authors obtained good results in the treatment of esthesioneuroblastoma with intracranial extension. Key words:Esthesioneuroblastoma;Intracranial extension;Chemotherapy.