Congenital cataract is the leading cause for children's blindness in most countries. Approximately one third of all the causes of Congenital cataract are familial and autosomal dominant blindness infants. The etiology of congen ital cataract is heterogenous. With the development of molecular biology techniques, researches on the mechanism of congenital cataract have made great progress. This review focused on the molecular mechanism of congenital cataract.