Background: Dysembryoblastic neuroepithelial tumors (DNETs) are benign tumors of the cerebral cortex that most commonly occur in children or young adults. Seizures are a frequent presenting feature, with an incidence of 80-100%, and are often an indication for surgical resection. Methods: We performed a retrospective chart review of children with DNETs who underwent epilepsy surgery between 1998 and 2014. Results: A total of 12 subjects were identified (6 males, 6 females), all of whom had seizures prior to surgical resection. Of these patients, 1 had infantile spasms, 2 had simple partial seizures and 10 had complex partial seizures. Tumors were located in the temporal (n=7), frontal (n=3) or parietal (n=2) cortex. These patients went on to have surgery on average 15 months after seizure onset, 3 had incomplete resections. At an average follow up of 6 years 4 months, all patients were class 1 on Engel’s Classification. All but one subject with rare non-disabling seizures were seizure free, with only 6 on medication. Follow up MR imaging revealed tumor recurrence in 1 subject. Conclusions: Despite differing seizure seminology and tumor location, surgical resection of these low-grade tumors resulted in excellent seizure outcome even in the setting of incomplete tumor resection.