Exceptionally, HCC has been seen in patients with APC germ-line mutations or presenting rare monogenic metabolic diseases such as hemochromatosis (HFE1 gene alterations), Wilson disease (ATP7B gene alterations), tyrosinemia type I (FAH gene alterations), cutanea tarda (UROD gene alterations), a1 antitrypsin disease (SERPINA1 gene alterations), von Gierke disease (G6PC gene alterations), or diabetes of the young type 3 (MODY3 or hepatocyte nuclear factor 1A; HNF1A gene alterations) [22,65]. This evidence concerns the gene HNF1A and tyrosinemia type I.