Combined hepatocellular-cholangiocarcinoma (cHCC-CC) is a rare primary liver malignancy composed of cells with histopathological features of both cholangiocarcinoma (CC) and hepatocellular carcinoma (HCC). It reportedly accounts for 0.4–14.2% of all primary liver carcinomas, with incidence varying in different regions. The clinical features are similar to those of either HCC or CC. Preoperative noninvasive diagnosis of cHCC-CC with conventional radiography is difficult. Because the origin of cHCC-CC is from two different tumour entities, despite intensive preoperative imaging studies, most studied patients were misdiagnosed either as HCC or CC. Accurate preoperative diagnosis is important because the most appropriate treatment depends on the major component of the tumour (HCC or CC). A high index of suspicion, imaging studies (ultrasound, computed tomography, positron emission tomography), levels of serum tumour markers (α-fetoprotein, carbohydrate antigen 19-9), and histology assist case detection and treatment choice. Patients who qualify for surgery should have a partial hepatectomy with hilar lymph node dissection, which can result in 5-year survival rates exceeding 50%. The role of liver transplantation is not yet known.