Abstract: Hepatoid adenocarcinoma (HAC) of the gallbladder is a variant of extrahepatic adenocarcinoma with hepatic differentiation. With similar clinicopathological presentation, the main differential diagnoses are hepatocellular carcinoma (HCC) and gallbladder carcinoma (GBC). At present, HAC of the gallbladder have only been reported in a few researches. This article will summarize comprehensive researches on the histological origin, clinical characteristics, histopathological and immuno-histochemical characteristics, differential diagnosis, therapy and prognosis of HAC of the gallbladder.