Ex vivo liver resection and autotransplantation (ELRA) represents a pioneering technique that provides a curative solution for complex hepatic diseases untreatable by conventional hepatectomy. This article systematically reviews the indications, key techniques, and clinical outcomes of ELRA. By utilizing ex vivo hypothermic perfusion, precision lesion resection, and vascular reconstruction, ELRA significantly expands surgical indications for benign and malignant diseases, such as hepatic alveolar echinococcosis (AE), hepatocellular carcinoma, and cholangiocarcinoma involving the hepatic hilum, hepatic veins or inferior vena cava, while avoiding donor shortages and immunosuppressive risks associated with allogeneic liver transplantation. Preoperative three-dimensional reconstruction and functional liver volume assessment, combined with intraoperative venous bypass-free techniques and outflow reconstruction strategies, enhance procedural safety. Postoperative anticoagulation therapy and monitoring of complications such as hepatic venous obstruction and biliary stricture, reduce mortality and severe morbidity. Studies demonstrated that ELRA achieved a 1-year survival rate of 89.7% in benign lesions and a >80% 5-year survival rate in end-stage hepatic AE, significantly outperforming non-surgical approaches. Future advancements in artificial intelligence navigation, minimally invasive techniques, and novel organ preservation solutions, may further optimize ELRA, establishing it as a standard intervention for complex hepatobiliary diseases.