Abstract: 【Abstract】Primary congenital glaucoma(PCG) occurs in the infants which can inherit and lead to children blindness. It is also the most common type of congenital glaucoma. Genetic factor is the main cause of the disease. Currently, the candidate genes of PCG are CYP1B1, LTBP2, and MYOC. The modes of inheritance of those genes, mutation screening, the gene structure and functional changes after mutation have been studying. However, the mutations of these genes can not fully explain the pathogenesis of PCG. Therefore, the researchers will focus on the new diseasecausing genes and mutation. (Int Rev Ophthalmol, 2012, 36: 293297)