Combined hepatocellular carcinoma (cHCC-CCA) is an uncommon primary liver lesion in which aspects of hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) are intimately and unequivocally expressed. Its origin is still a topic of debate, given its heterogenicity, molecular profile, gene mutations but also to the complexities of the microenvironment in which it develops and to the pathways that are triggered in the liver in relation to the phenomena of liver damage. Diagnosis and treatment are still a challenge.