We describe a dual pathology presenting as intractable temporal lobe epilepsy associated with subcortical ectopic gray matter. The patient was a 28-year-old male with a 12-year history of refractory temporal lobe epilepsy. Preoperative diagnostic imaging revealed right hippocampal sclerosis, in addition to subcortical ectopic gray matter extending from the posterior end of the inferior horn of the right lateral ventricle to the cerebral parenchyma in the temporoparietal lobe. As surgical therapy for epilepsy, right anterior temporal lobectomy with amygdalohippocampectomy was initially performed. Intraoperative electrocorticography (ECoG) was extremely useful at this point in determining the range of excision of ectopic gray matter after resection of mesial temporal lobe structures. Based on ECoG findings, about 50% of the ectopic gray matter was excised. As of 2 years postoperatively, the patient has remained seizure free with no medication. In cases concomitantly manifesting hippocampal sclerosis and subcortical ectopic gray matter, epilepsy may be associated with dual pathology. This case report raises the potentially important issue of the possible presence of areas of structural abnormality that are non-epileptogenic.