Documentation of familial epilepsy is of paramount importance for identification of epilepsy-associated genes, elucidation of pathomechanisms of epilepsy, and development of treatment of epilepsy. We report a Japanese family with 5 members with lateral temporal lobe epilepsy beginning around the second decade of life. All seizures were intractable to medical treatment, and four patients underwent surgical treatment following long-term monitoring by intracranial electroencephalography with subdural electrodes, which revealed neocortical origins for the seizure. These four patients were successfully treated with surgery. The clinical features of this familial temporal lobe epilepsy seem to be different from those of previously reported types of familial temporal lobe epilepsy.