ObjectiveTo summarize the diagnosis and treatment progress of combined hepatocellular-cholangiocarcinoma (cHCC-CCA) in recent years, in order to provide a reference for clinical diagnosis and treatment decision-making. MethodThe recent literature related to the diagnosis and treatment of cHCC-CCA was reviewed. ResultsThere was no specific guideline or consensus on the diagnosis and management of cHCC-CCA. The diagnosis of the cHCC-CCA was challenging by imaging alone, and the tumor markers such as alpha fetoprotein and carbohydrate antigen 19-9 were of particular value in clinical diagnosis, but it was ultimately relied on the pathological examination results. For the patients with early cHCC-CCA, the radical surgery was recommended to undergo if possible, but there was some controversy regarding the efficacy of liver transplantation and the mode of recurrence and metastasis. For the patients with advanced cHCC-CCA, the systemic therapy was being explored further, and some retrospective analyses of small samples suggested that the gemcitabine and platinum-containing chemotherapy regimens might be beneficial. With the better success of immunotherapy and targeted therapies in hepatocellular carcinoma and cholangiocarcinoma, it might provide some experiences for treatment selection of cHCC-CCA. ConclusionscHCC-CCA has a lower incidence rate and stronger heterogeneity. Its diagnosis mainly relies on surgical pathology, and treatment strategy is lack of high-level evidence-based medical evidence and rigorously designed clinical studies are still needed to explore its efficacy and safety in the future.