Primary hematolymphoid malignancies of the extrahepatic biliary tract are rare tumors. We report five cases of primary hematolymphoid malignancies involving the extrahepatic biliary tract. One is a granulocytic sarcoma of the extrahepatic bile duct, another is an extramedullary plasmacytoma of the gallbladder, and the others are two non-Hodgkin lymphomas of the extrahepatic bile duct and one of the gallbladder. The clinical presentations, radiographic studies, and gross findings at surgery have not been a significant help in differential diagnosis. Although a preoperative diagnosis of primary hematolymphoid malignancy is very difficult to reach because of the rarity of this disease, it should be considered, because, if an accurate diagnosis is made before surgical intervention, chemotherapy is the most appropriate treatment. In limited cases mimicking cholangiocarcinoma and gallbladder cancer, surgical resection followed by chemotherapy has a valid role as reasonable treatment for patients.