To assess the efficacy of intravenous immunoglobulin (IVIG) as add-on treatment for intractable cholangitis (IC) after Kasai portoenterostomy (KPE) in biliary atresia (BA) patients. 113 BA patients who had one or more episodes of cholangitis after KPE were recruited in this study. According to whether response to routine conservative treatment, all patients were divided into IC group and simple cholangitis (SC) group. Meanwhile, patients with IC subdivided into IVIG group and control group according to whether application of IVIG. The IC group had higher serum procalcitonin (PCT) (P = 0.014), C-reactive protein (CRP) (P = 0.023), and gamma-Gltamyltranspeptidase (gamma-GGT) (P = 0.031) level than the SC group. The IVIG group had shorter duration of fever after treatment (P = 0.011) and length of hospital stay (P = 0.018) than the control group. The time until recurrent episode of cholangitis was significant longer in IVIG group than in control group (P = 0.019). IVIG as add-on treatment may be an effective treatment for the cholangitis acute episode, and we conclude by calling for more prospective studies to attest to the role of IVIG in the treatment of cholangitis.