Neoplasms are a well-established cause of medically intractable or chronic epilepsy. Certain tumors, including gangliogliomas and dysembryoplastic neuroepithelial tumors, are well known to be associated with cortical dysplasia. This study retrospectively examines the incidence of coexistent pathology in patients with tumors and chronic epilepsy. This study is a retrospective review of 270 tumors arising in patients with medically intractable epilepsy encountered during a 20-year period (1989-2009). Coexistent pathology was noted in 50 of 270 (17.8%) patients, including 27 males (54%) with a mean age at surgery of 18 years (range 1-52 years). The vast majority of lesions (n=40) (80%) were located in the temporal lobe and less commonly in the parietal lobe (n=4) and the occipital lobe (n=3). Tumor diagnoses included ganglioglioma (n=29), dysembryoplastic neuroepithelial tumor (n=10), low-grade glial/glioneuronal neoplasm (n=5), low-grade astrocytoma (n=2), angiocentric glioma (n=1), low-grade mixed glioma (n=1), dysembryoplastic neuroepithelial tumor/ganglioglioma mixed tumor (n=1), and meningioangiomatosis (n=1). Forty-one (82%) tumors represented WHO grade-I neoplasms. Concomitant pathology included malformation of cortical development (cortical dysplasia) in 40 patients (80%) (Palmini et al type-I: n=37; Palmini et al type-II: n=3). Hamartias were identified in 10 patients (20%), hippocampal sclerosis in four patients (8%), and nodular heterotopia in one patient (2%). The true incidence of coexistent pathology (17.8% in this study) was likely underrepresented, given the limited extent of adjacent non-tumoral tissue sampling in cases of resected tumor. Coexistent pathology may account for the incidence of recurrent or residual epilepsy in patients who undergo tumor resection.