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The medical charts of 162 patients who had been subjected to brain tumor surgery between 1997 and 2005 in the Institute of Neurology Deolindo Couto were reviewed. Of the 162 patients subjected to cranial intervention, 5 (3.8%) had presented criteria for the dysembryoplastic neuroepithelial tumor. Two patients had tumor in the frontal lobe, 2 in the parietal lobe, and 1 in the temporal area. The age at seizure onset ranged from 2 to 17 years. The average duration of active epilepsy preoperatively was 4 to 22 years. A review of the literature was carried out and the histologic and radiologic aspects were commented upon. Correct histologic diagnosis is important, because dysembryoplastic neuroepithelial tumor presents an excellent prognosis, and they can be confused with other tumors.