Congenital ectopic lentis is defined as a kind of connective tissue disease with obvious genetic predisposition that lens dislocates caused by imbalance of the traction of lens as a result of zonular fiber defect or dysplasia. We get further understanding of the molecular etiology and nosogenesis of congenital ectopic lentis under guidance of scientific research in recent years.Main suspicious disease-causing genes will be reviewed in this paper to provide reference for clinical diagnosis and treatment.