Abstract: In this study, 46 patients with Febrile Seizures were selected and total DNA was extracted from white blood cells. All exons of GABRG2 gene were amplified by standard PCR. After which the PCR products were screened by Single Stranded Conformational Polymorphism (SSCP) analysis. Selected samples in SSCP analysis that showed new conformer were sequenced. In order to verify the sequencing data, a restriction analysis was carried out. We report R82Q mutation in GABRG2 gene in an Iranian family. Proband was a 12 years old boy. SSCP and Sequencing of exon-2 of GABRG2 gene in proband demonstrated an exonic G245A mutation leading to R82Q substitution. The RFLP of PCR product showed heterozygosity in patients. Arg82 is a conserved amino acid in the benzodiazepine binding site at the N-terminus of the g2 subunit. The R82Q mutation does not alter the response of the receptor to Gamma Amino Butyric Acid but impairs the potentiation by benzodiazepine. Result of this research and several other lines of evidence support complex inheritance, heterozygosity and diverse phenotypes for epilepsy.