Hereditary Tyrosinemia type 1 (HT-1), also known as hepatorenal Tyrosinemia, [1] is an inborn error of metabolism of autosomal recessive inheritance, caused by mutations in the fumarylacetoacetate hydrolase (FAH) gene located in the 15q23-q25 locus that encodes the fumarylacetoacetate hydrolase enzyme involved in tyrosine catabolism [2], which leads to the accumulation of toxic metabolites such as succinylacetone and maleylacetoacetate, [3] which causes different conditions such as severe liver cirrhosis, liver failure, neurological crises and even hepatocellular carcinoma [4]. Here, FAH is linked to hepatocellular carcinoma.