Peripheral cholangiocarcinoma is a relatively rare cancer. However, it is known to have an unfavourable prognosis compared with that of hepatocellular carcinoma. Little is known about its aetiology, clinical or pathological features. Recently, with the development of imaging modalities, early staged cholangiocarcinoma has been diagnosed with relative ease. Surgery is the optimal therapy. Total hepatectomy does not provide survival benefit. Conventional surgery remains the only effective treatment, even for patients with advanced-stage tumours. Factors influencing survival after hepatectomy were tumour-free margin, lymphnodes metastasis and histopathology of tumour. Palliative intrahepatic tubing or percutaneous transhepatic biliary drainage and brachytherapy can alleviate jaundice and cholangitis, thereby prolonging survival in some cases.