BACKGROUND: The impact of transjugular intrahepatic portosystemic shunt misplacement on outcomes of liver transplantation remains controversial. We systematically reviewed the literature on the outcomes of liver transplantation with transjugular intrahepatic portosystemic shunt misplacement. METHODS: This systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The Cochrane library, PubMed, and Embase were searched (January 1990-April 2020) for studies reporting patients undergoing liver transplantation with transjugular intrahepatic portosystemic shunt misplacement. RESULTS: Thirty-six studies reporting 181 patients who underwent liver transplantation with transjugular intrahepatic portosystemic shunt misplacement were identified. Transjugular intrahepatic portosystemic shunt was misplaced with a variable degree of extension toward the inferior vena cava/right heart in 63 patients (34%), the spleno/portal/superior mesenteric venous confluence in 105 patients (58%), and both in 15 patients (8%). Transjugular intrahepatic portosystemic shunt thrombosis was also present in 21 cases (12%). The median interval between transjugular intrahepatic portosystemic shunt placement and liver transplantation ranged from 1 day to 6 years. Complete transjugular intrahepatic portosystemic shunt removal was successfully performed in all but 12 (7%) patients in whom part of the transjugular intrahepatic portosystemic shunt was left in situ. Cardiac surgery under cardiopulmonary bypass was necessary to remove transjugular intrahepatic portosystemic shunt from the right heart in 4 patients (2%), and a venous graft interposition was necessary for a portal anastomosis in 5 patients (3%). Postoperative mortality (90 days) was 1.1% (2 patients), and portal vein thrombosis developed postoperatively in 4 patients (2%). CONCLUSION: Misplaced transjugular intrahepatic portosystemic shunt removal is possible in most cases during liver transplantation with extremely low mortality and good postoperative outcomes. Preoperative surgical strategy and intraoperative tailored surgical technique reduces the potential consequences of transjugular intrahepatic portosystemic shunt misplacement.