Abstract: Objectives　To investigate clinical and genetic characteristics of one pair of monozygotic twins with epilepsy combined with intellectual disability. Methods　Clinical characters and genetics analysis of monozygotic twins with  epilepsy combined intellectual disability were retrospectively analyzed. With "FLNA" as term for searching literature through PubMed, single nucleotide polymorphism database, human genome mutation database and Online Mendelian Inheritance in Man. Results　A pair of 3 years and 3 months old monozygotic twins with complaint of recurrent seizures, development delay and intellectual disability. Laboratory findings including increased neuron-specific enolase in serum, epileptic waves found by video-EEG and widened extracranial space by brain MRI. Gene detection revealed a hemizygous missense mutation c.7568G>A (p.Ser2523Asn) in FLNA gene, which has not been reported. Conclusions　When patient presented with repeated seizures with varied degrees of intellectual disability and brain dysplasia, it is necessary to screen FLNA gene mutations to help diagnosis. Our report expanded FLNA gene mutation spectrum. .