We described a female patient with epilepsy due to protocadherin 19 (PCDH19) non sense mutation (p. Y280X). Her parents had no mutation. There are no patients with epilepsy or mental retardation in her families. She had cluster seizures associated fever from 9 months old. She had moderate mental retardation and autistic tendency. The seizure clusters became worse by carbamazepin. Although monthly fever episodes have been persisted, seizures disappeared after administration of clorazepate at 12 years of age. In females having recurrent episodes with fever associated cluster seizures, PCDH 19 analysis should be considered.
