Congenital biliary atresia is considered a neonatal rare disease with multiple causes and phenotypes,whose pathogenesis remains unclear. At present,the etiological study of congenital biliary atresia mainly focuses on viral infection,immune disorder,and dysplasia.This paper summarizes the main etiological hypotheses about congenital biliary atresia nowadays and the connections between them. The research progress provides valuable clues and evidence for the etiology and control of this disease.