A 26-year-old man presented with a case of angiocentric glioma manifesting as medically refractory epilepsy. Magnetic resonance imaging revealed a hyperintense lesion in the right superior frontal gyrus on T(2)-weighted imaging, with cortical hyperintense rim on T(1)-weighted images and minimum contrast enhancement. Video-electroencephalography (EEG) monitoring characterized his seizures as originating from the right frontal lobe. Long-term EEG recording from implanted subdural electrodes disclosed epileptic activities extending beyond the margin of the radiological lesion. Extended cortical resection of the superior frontal gyrus including the tumor and the surrounding epileptic cortices was performed. Postoperatively, he became seizure-free with antiepileptic medication during a 12-month follow-up period. Histological examination of the surgical specimen showed the characteristic findings of angiocentric glioma. Associated cortical dyslamination consistent with cortical dysplasia was found in the surrounding cortex. Angiocentric glioma is a slow-growing or stable tumor frequently presenting with intractable epilepsy. Surgical treatment would be aimed primarily at control of epilepsy. Complete lesionectomy usually results in postoperative seizure freedom, but the present case shows evidence for associated cortical dysplasia with this tumor entity. Careful pre-surgical evaluation for epilepsy is necessary to achieve better seizure outcome.