Epilepsy is a common disease in children's nervous system diseases. With the application of gene sequencing technology in clinical practice， more and more children are found to be genetic associated epilepsy. γ-Aminobutyric acid type A receptor （GABAAR） is a ligand-gated chloride channel， which mediates the main inhibitory function of the central nervous system. Its dysfunction plays an important role in the etiology of epilepsy. It has been confirmed that the subunits of GABAAR including GABRA1， GABRA6， GABRB2， GABRB3， GABRG2 and GABRD are related to epilepsy. In this paper， we review the mechanism and clinical phenotype of the GABAAR subunit related epilepsy for the precise treatment of these patients.