The majority of tumors that present with epilepsy comprise a group of tumors that include dysembryoplastic neuroepithelial tumors (DNT), ganglioglioma, gangliocytoma, and the more recently described angiocentric glioma. This chapter focuses on these tumors and their management is predominantly focused on seizure control. The differential diagnosis includes other low-grade epilepsy-associated tumors, cortical dysplasia, and diffuse gliomas. The chapter discusses the lesions which are distinguished from the dysplastic gangliocytoma of the cerebellum and desmoplastic infantile ganglioglioma. Ganglioglioma accounts for up to 50% of all tumors in patients undergoing surgery for control of epilepsy and is the commonest tumor associated with chronic epilepsy. The goals of management are seizure control, confirmation of the radiological diagnosis, and control of tumor growth and progression. Angiocentric glioma is a low-grade cortical tumor associated with epilepsy. The differential diagnosis includes low-grade glioneuronal tumors, focal cortical dysplasia (FCD), and diffuse glioma.