A 27-year-old Tibetan woman from the pastoral area of Qinghai Province was admitted to our hospital without any discomfort. Physical examination revealed liver-occupying lesions in August 2017. On abdominal contrast-enhanced computed tomography and magnetic resonance imaging, a 111 × 15 × 10 mm3-sized irregular lesion was found in the right third lobe and caudate lobe of the liver, extending to the right adrenal gland and invading the diaphragm, inferior vena cava (IVC), and hepatic hilus (Figure A: 1-4). On IVC venography, the vein was completely blocked and collateral circulation such as the azygos and hemi-azygos veins was well compensated (Figure A: 5 and 6). The WHO-IWGE PNM classification system was P4N1M0 phase1,2. We performed three-dimensional reconstruction and virtual excision of the liver before operation, using IQQA-Liver (EDDA technology, USA; Figure B: 1 and 2). Hepatic right third lobe and caudate lobectomy combined with retrohepatic IVC resection was performed. We had to perform a stepwise resection because the large lesion limited the operating space (Figure C: 1-3). We decided to ligate and cut the blocked IVC between 1 cm below and above the confluence of the right and left hepatic veins, respectively (Figure C: 4 and 5). Postoperative pathological results confirmed hepatic alveolar echinococcosis (HAE) and an invaded IVC (Figure B: 3 and 4). Enlarged lymph nodes were removed during the operation, and postoperative pathological results indicated lymph node reactive hyperplasia (Figure B: 5). Approximately 70% of HAE lesions are located in the right lobe, and 40% encroach the hepatic hilus and rarely infringe the IVC. Surgery is the first-choice treatment for HAE, but radical surgery is performed only in 35% of patients, especially when the first and second hepatic portals are involved. Although combined liver and IVC resections involve a considerable risk, curative surgical resection could improve the long-term survival of patients with invaded IVCs.