In order to elucidate the radiological features of dysembryoplastic neuroepithelial tumor (DNT), and to clarify the optimal surgical strategy for this tumor, the authors retrospectively analyzed 20 cases of DNT treated at our institution. Magnetic resonance (MR) imaging (all cases), 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) (eight cases), ictal/interictal Tc99m-HMPAO single photon emission computed tomography (SPECT) (seven and five cases respectively) and proton magnetic resonance spectroscopy (1H MRS) (one case) were performed preoperatively. Invasive monitoring/intraoperative electrocorticography (ECoG) was performed in four cases in order to determine the epileptogenic zone. A well-demarcated lobulating tumor located in the cortical with/without subcortical area was the typical MR finding. 18F-FDG PET showed glucose hypometabolism in all cases. Ictal Tc99m-HMPAO SPECT showed hyperperfusion of the lesion in three cases and interictal Tc99m-HMPAO SPECT showed hypoperfusion of the lesion in one case. 1H MRS showed nonspecific findings. Gross total resection was performed in all cases. Histologically, associated cortical dysplasia was found in 11 cases. The mean duration of follow-up after surgery was 37.9 months, and the overall seizure free rate was 90%. Follow-up MR imaging was performed in 14 cases (mean duration of follow-up: 21.6 months) and showed no recurrence of tumor in any of these cases. Invasive monitoring/intraoperative ECoG and the presence of cortical dysplasia showed no significant relationship with seizure control rate (p = 1.25 and p = 1.62 respectively).