In this chapter, we describe the embryonic development of the liver and the developmental anomalies (otter-tailed liver, hepatic parietal lobe, and congenital portosystemic shunt) as well as their imaging manifestations and differential diagnostic points. In addition, we present the clinical and imaging features of benign diseases (hepatic glycogen storage disease, hepatic cavernous hemangioma, hepatic mesenchymal malformation, and hemangioendothelial cell tumor) and malignant disorders (hepatoblastoma, hepatic undifferentiated embryonal sarcoma, and hepatic lymphoma) of the liver in children, respectively, with a special emphasis on the key points of diagnostic imaging. At the end of the chapter, we present knowledge about common diseases of the biliary tract in children (biliary dysplasia, congenital biliary atresia, and congenital dilatation of the bile ducts), including clinical and imaging and differential diagnostic elements.