OBJECT: Dysembryoplastic neuroepithelial tumors (DNETs) are a subset of relatively rare glioneuronal tumors that typically present with epilepsy during childhood. The authors' aim was to identify factors that predict seizure control following excision. METHODS: The authors reviewed the cases of 50 patients who underwent resection of DNETs at the University of California, San Francisco, between 1990 and 2006. Demographic, seizure history, radiographic, and histopathological data were collected and analyzed for statistical association with postoperative seizure control. RESULTS: Of the 50 patients, 86% presented with intractable epilepsy. The median age at surgery was 21 years (range 4-46 years; 40% were < 18 years old at time of surgery), with a median duration of 8 years from onset of seizures (24% were adult-onset seizures). Fifty-two percent of the cases were associated with adjacent focal cortical dysplasia. Complete resection was achieved in 78% of cases. Intraoperative electrocorticography in 23 patients identified extralesional interictal activity in 16 cases, which led to extended lesionectomy or lobectomy. The remaining patients underwent lesionectomy alone. The median follow-up was 5.6 years, during which time tumor progression occurred after subtotal resection. The proportional estimates of seizure freedom (Engel Class I outcome) were 0.86 at 1 year and 0.85 at 5 years. Seizure freedom was predicted by complete or extended resection (OR 1.68, 95% CI 1.39-2.03; p < 0.0001) and extratemporal location (OR 1.20, 95% CI 1.02-1.42; p = 0.03) on multivariate analysis. Secondary analysis for intraoperative electrocorticography cases demonstrated that seizure outcome was better when extralesional spiking foci were detected (94% seizure free) compared with when they were absent (43% seizure free). CONCLUSIONS: Excision of DNETs and, when present, adjacent dysplastic cortex was highly effective for seizure control. Excellent seizure-free outcomes and tumor control were seen with lesionectomy alone in most cases. Electrocorticography with extended resection was useful for patients with pharmacoresistant epilepsy.