Hepatoid adenocarcinoma is a rare neoplasm, which has a striking morphologic similarity to hepatocarcinoma. It has been described in different organs, the most common are stomach, lung, and pancreas. In some cases, it is characterized by high serum levels of α-fetoprotein. This tumor has a pattern similar to the hepatocarcinoma. The typical features are a combination of histopathologic aspects of solid nests and trabecular structures of polygonal atypical cells with eosinophil and granular cytoplasm and immunohistochemical expression of α-fetoprotein and of carcinoembryonic antigen in half of cases. Here, we report the case of an old female patient affected by hepatoid adenocarcinoma of the ureter with ovarian, small intestine, and hepatic involvement. We discuss the clinical aspects, the morphologic features, and the immunoistochemical staining useful for differential diagnosis.