Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare inherited metabolic disorder caused by loss-of-function mutations in the nuclear gene TYMP leading to thymidine (Thd) and deoxyuridine (d-Urd) accumulation [1]. Here, TYMP is linked to mitochondrial neurogastrointestinal encephalomyopathy.