Background: Biliary atresia (BA) is the leading cause of pediatric liver failure and pediatric liver transplantation worldwide. Evidence suggests that the immune system plays a central role in the pathogenesis of BA. Methods: In this work, the novel immune-related genes between BA and normal samples were investigated based on weighted gene co-expression network analysis (WGCNA) and the deconvolution algorithm of CIBERSORT. Results: Specifically, 650 DEGs were identified between the BA and normal groups. The blue module was the most positively correlated with BA containing 3274 genes. Totally, 610 overlapping BA-related genes of DEGs and WGCNA were further used to identify IRGs. Three IRGs including VCAM1, HLA-DRA, and CD74 were finally identified as the candidate biomarkers. Particularly, the CD74 biomarker was discovered for the first as a potential immune biomarker for BA. Conclusions: Possibly, these 3 IRGs might serve as candidate biomarkers and guide the individualized treatment strategies for BA patients. Our results would provide great insights for a deeper understanding of both the occurrence and the treatment of BA.