Fumarylacetoacetate hydrolase (FAH) plays a key role in tyrosine metabolism to produce succinylacetone; and FAH deficiency mainly induced by a heredity problem of FAH encoded gene leads to type 1 tyrosinemia, in which increased levels of succinylacetone are observed along with liver diseases including hepatic failure, LC, and liver cancer (Scott, 2006; Yang et al., 2017). Here, FAH is linked to hyperinsulinemic hypoglycemia, familial, 4.