Abstract
Mitochondrial neurogastrointestinal encephalopathy (MNGIE) is a rare, complex mitochondrial disorder with variable phenotypes caused by a defect in the TYMP gene that codes for the thymidine phosphorylase enzyme. Orthotopic liver transplantation (OLT) has been proposed as a curative option for patients by using the liver as a source to restore thymidine phosphorylase levels in the body. Anesthetic considerations for this syndrome have not been clearly outlined in the past. We describe the clinical presentation of a young woman with MNGIE, her perioperative assessment, and intraoperative management during liver transplantation.