Abstract: Biliary atresia (BA) is characterized by progressive inflammatory obstruction of intrahepatic and extrahepatic bile ducts and hepatic fibrosis, which is one of the common causes of obstructive jaundice in infants. Kasai surgery is the first choice for children with BA. Timely and effectively relieving inflammatory obstruction of bile duct and curbing liver fibrosis are the key to prolong or realize autologous liver survival in children with BA. GATA binding protein 6 (GATA6) has the function of regulating transcriptional activity. Studies have shown that it can inhibit the activation of hepatic stellate cells (HSCs) by regulating cell proliferation and differentiation, up-regulating the expression of ferritin and participating in mitogen-activated protein kinase-dependent signal pathway, promote HSCs senescence, reduce liver fibrosis, and play a certain role in the process of liver repair after BA liver injury. This article intends to review the relationship between GATA6 and BA liver fibrosis from the aspects of functional characteristics and pathways of action.