Background For patients with end-stage hepatic alveolar echinococcosis (AE), in vivo resection of the involved parts of the liver is usually very difficult, therefore, allogenic liver transplantation is indicated. However, we hypothesize that for selected patents, ex vivo liver resection for thorough elimination of the involved tissues and liver autotransplantation may offer a chance for clinical cure.Methods We presented a 24-year-old women with a giant hepatic AE lesion who was treated with hepatectomy, ex vivo resection of the involved tissue and hepatic autotransplantation. The patient had moderate jaundice and advanced hepatic AE lesion which involved segments Ⅰ, Ⅳ, Ⅴ, Ⅵ, Ⅶ, Ⅷ and retrohepatic inferior vena cava. The lateral segments (Ⅱ and Ⅲ) of the left liver remained normal with over 1000 ml in its volume. No extrahepatic metastases (such as to the lung or brain) could be found. As the first step of treatment, X-ray guided percutaneous transhepatic cholangiodrainage (PTCD) was performed twice for bile drainage in segment Ⅲ and Ⅱ separately until her serum total bilirubin decreased gradually from 236 to 88 μmol/L. Total liver resection was then performed, followed by extended right hepatic trisegmentectomy and the entire retrohepatic vena cava was surgically removed en bloc while her hemodynamics parameters were stable. Neither veino-veinous bypass nor temporary intracorporeal cavo-caval or porto-caval shunt was used during the 5.7-hour anhepatic phase. The remained AE-free lateral segments of the left liver were re-implanted in situ. The left hepatic vein was directly anastomosed end-to-end to the suprahepatic inferior vena cava due to the lack of the retrohepatic inferior vena cava with AE total infiltration. Because compensatory retroperitoneal porto-caval collateral circulation developed, we enclosed remained infrahepatic inferior vena cava at renal vein level without any haemodynamics problems.Results During a 60-day following-up after operation, the patient had a good recovery except for a mildly elevated serum total bilirubin.Conclusions As a radical approach, ex vivo liver resection and liver autotransplantation in a case has shown a optimal potential for treatment of the end-stage hepatic AE. Strict compliance with its indications, evaluation of vessels of patients pre-operatively, and precise surgical techniques are the keys to improve the prognosis of patients.