The key consideration when performing living donor liver transplantation (LDLT) in patients with Budd-Chiari syndrome (BCS) is careful management of a stenotic or occluded inferior vena cava (IVC), because it is not possible to replace the recipient stenotic or occluded IVC with donor IVC as in cadaver donor transplantation. We describe how we performed LDLT with extensive thrombectomy in a patient with acute-on-chronic BCS with a totally thrombosed retrohepatic IVC. The operation was successful and the patient remains well, with follow-up images showing a patent IVC and hepatic veins. To our knowledge, LDLT for a BCS patient with severe extensive caval thrombus has never been reported before. We consider that the successful outcome of this patient clearly demonstrates the feasibility of our technique of extensive thrombectomy, without a vessel graft, to manage a stenotic or occluded IVC in LDLT in patients with BCS.