Transjugular intrahepatic portosystemic shunt (TIPS) is a minimally invasive procedure used for the decompression of portal pressure since the early 1990s. Bacteremia with an infection of the TIPS or endotipsitis is a serious complication with 32% mortality. To date and including the present case, only 56 cases have been reported in the literature. There are concerns regarding underdiagnosis. We report a case of a patient with endotipsitis and recurrent bacteremia treated successfully in our institution. In addition, previous reported cases were reviewed regarding microbiological data and mortality. Clinical practice guidelines including uniform disease definition, prophylaxis, and treatment of this nosocomial prosthetic-related infection are urgently needed.