We report a 30-year-old man with temporal lobe epilepsy associated with hypocalcemia due to primary hypoparathyroidism. He had frequent jamais vu since age 14 years, and later developed generalized convulsive seizures. It was initially controlled by phenytoin. The patient also had hypocalcemia due to primary hypoparathyroidism. The presence of jamais vu suggested the seizure focus in the mesial temporal area, being consistent with EEG finding, and it was most likely activated by the associated hypocalcemia. Control of hypoparathyroidism, and furthermore, selection of valproic acid as an antiepileptic drug which did not interfere with calcium metabolism were essential for this particular patient.