Background: Clinical and neuroimaging features of patients with epilepsy and coexisting extratemporal porencephaly and hippocampal sclerosis have been previously described. Objective: To present the clinical characteristics and surgical outcome of 6 patients with intractable epilepsy and coexisting extratemporal porencephaly and hippocampal sclerosis. Patients and Methods: Twenty-four patients with porencephaly and epilepsy were studied. Of these, 6 had an epileptogenic focus in the temporal region. All patients underwent video electroencephalogram monitoring, magnetic resonance imaging studies, and neuropsychological evaluation. Of the subset of patients with temporal lobe epilepsy, 1 patient underwent intracranial electroencephalogram monitoring. Temporal lobe resection was performed in 5 patients. Outcomes were evaluated using the Engel classification. Results: Freedom from seizures was achieved in all patients. Pathologic analysis of the resected tissue confirmed the presurgical diagnosis of mesial temporal sclerosis. Conclusion: Patients with extratemporal porencephaly and intractable seizures should be evaluated early and be considered for temporal lobectomy if clinical, magnetic resonance imaging, and electroencephalogram findings support the diagnosis of temporal lobe onset seizures.