Portal venous thrombosis was originally considered to be a contraindication for liver transplantation. Currently, several methods exist to re-establish blood flow to the hepatic portal system. Cavoportal hemitransposition is a surgical procedure that can be used in liver transplantation when the portal venous system is thrombosed and portal flow cannot be re-established from the mesenteric venous system. In cavoportal hemitransposition the blood flow from the inferior vena cava of the recipient is directed to the portal vein of the donor liver to compensate for the lost portal venous supply. This can either be done by end-to-end or end-to-side anastomosis. Seventy-one cases of cavoportal hemitransposition have been reported worldwide. All patients reported had been in a critical and life-threatening condition, presenting with either end-stage-liver disease or acute hepatic failure combined with severe vascular pathology. Of the cases reported, 32 patients died for reasons non-related to the surgical procedure. Seven of the 71 patients had Budd-Chiari syndrome complicated by thrombosis of the portal-venous system. This means thrombosis in two different venous systems at the same time, the mesenteric and main venous system. To date this venous thrombosis of both the caval and portal system has only been reviewed together with the possible medical, radiological and non-transplant treatment options. This article evaluates the available literature and reviews the evolution of the surgical procedure of cavoportal hemitransposition emphasising its role in combined thrombosis of the hepatic veins and portal venous systems.