Objective To determine the clinical effects of type Ⅲ biliary atresia treated by hepatojejunostomy (Kasai procedure).Methods A retrospective study was performed on patients with type Ⅲ biliary atresia treated in our hospital between January 2009 to December 2013.Results The follow-up data were completed in 42 patients out of 48 patients (87.5％) who underwent Kasai procedure for type Ⅲ biliary atresia.The jaundice disappeared in 31 of 42 patients (73.8％).The rate of patients having cholangitis was 47.6％.There were significant differences in the 2-year survival among the groups of patients with mild cholangitis (75.0％,9/12),serious cholangitis (25.0％,2/8) and without cholangitis (86.4％,19/22) (P ＜ 0.05).Conclusions Hepatojejunostomy (Kasai procedure) is an effective operation to treat type Ⅲ biliary atresia.Our data suggested that the survival rates were related to the degree of bile drainage and cholangitis post surgery.