Rare liver diseases in pregnancy, including Wilson disease, Budd-Chiari syndrome, hereditary hemorrhagic telangiectasia (HHT), autoimmune hepatitis, primary biliary cholangitis (PBC), and hepatic adenomatosis, present complex challenges requiring specialized multidisciplinary management. Although individual incidences range from 1 in 10,000 to 1 in 100,000 pregnancies, these conditions collectively represent a significant cause of maternal morbidity and mortality, with rates historically ranging from 5% to 30%. The physiological hemodynamic changes of pregnancy—including increased cardiac output, expanded blood volume, and a hypercoagulable state—can exacerbate underlying pathology, precipitating acute decompensation such as liver failure, hemorrhage, or thrombosis. Acute surgical challenges in this population include emergency hepatic resection for ruptured adenomas, damage control surgery for hepatic hemorrhage, and management of vascular emergencies. Liver transplantation remains a definitive but high-risk intervention for acute liver failure, with maternal survival rates of 70–85% reported when intervention is timely. Multidisciplinary teams comprising hepatologists, transplant surgeons, maternal-fetal medicine specialists, and intensivists are essential for optimizing outcomes. While maternal survival is prioritized, fetal outcomes have improved, with survival rates between 40% and 70% largely dependent on gestational age at delivery and the severity of maternal illness. Future directions focus on improved genetic screening, non-invasive risk stratification, and the development of pregnancy-safe therapeutics to further reduce morbidity in this high-risk population.