Aim: To provide a fundamental understanding of the anatomical and histological structure of the liver in children with BA, which are essential for accurate diagnosis, the development of targeted treatment approaches, and effective surgical correction. Materials and Methods: A literature review was conducted on embryogenesis, anatomy, and morphology of the disease in newborns and children with biliary atresia (BA). At least 27 scientific sources were analyzed, covering the period from classical descriptions (since 1972) to modern publications (2020s). The selection criteria included studies describing liver embryogenesis, morphological features, and clinical and surgical aspects of BA. The databases PubMed, Medline, Embase, and Google Scholar were searched, covering research areas such as liver anatomy, physiology, and morphology in children, as well as clinical issues related to biliary atresia. In addition, the author’s observations were included. These were obtained during diagnostic imaging procedures, intraoperative evaluation of the organ during surgical correction, anatomical studies of 7 deceased children with BA, and histological examination of liver tissue. All patients were under our observation during diagnosis and treatment for BA and demonstrated characteristic clinical, anatomical, physiological, and morphological changes of the liver. Conclusions: The cause of BA may lie in disruptions or arrest of the embryonic development of the bile ducts and hepatic vascular system. In infants, the liver is in a state of anatomical immaturity and functional insufficiency, and its structure is not fully developed. This must be taken into account when treating children with BA.The anatomical differences in the liver of children with BA have significant practical implications for the surgical management of BA.