Klatskin’s tumor (or hilar cholangiocarcinoma) is an extra-hepatic cholangiocarcinoma arising in the junction of the main right and left hepatic bile ducts. Cholangiocarcinoma is a rare malignant neoplasm and accounts for only 3% of gastrointestinal tumours. Hilar cholangiocarcinoma is most common among them (50%), while distal and intrahepatic occur in fewer cases. Klatskin's tumor usually occurs at older age and it’s typically reported in patients older than 60 years. An exeption of the age of occurrence is found in patients with primary sclerosing cholangitis. In this group neoplasms occur earlier, typically within 2.5 years of diagnosis. In addition to the primary sclerosing cholangitis, there are several established risk factors including hepatolytosis, bile stasis, recurrent subclinical cholangitis, parasitic infections, and congenital anomalies of the biliary tract. In most patients, the disease is asymptomatic for a long time and if the symptoms occur they are non-specific and include icterus, light stool, dark urine, pruritus and weight loss. Techniques used to diagnose Klatskin’s tumor include abdominal imaging by computed tomography (CT) or magnetic resonance imaging (MRI) to assess for vascular invasion and provide noninvasive cholangiography. Furthermore, chest CT is obtained to exclude metastatic disease as well as laboratory testing including liver function tests and tumor markers CA 19-9 and CEA. The three systems most commonly used to evaluate hilar cholangiocarcinoma are the Bismuth-Corlette system, the MSKCC and the TNM classification. Surgical treatment is the only therapeutic modality that gives patients a chance for long-term survival. Depending on localization and type of tumor, surgical procedure may include local excision, aggressive resection or liver transplantation. Liver transplantation is a relatively new potential therapeutic method for patients with unresectable disease. Strict patient selection and preoperative neoadjuvant therapy following the Mayo Clinic protocol provide hope for possible recovery and outcome improvement in selected patients.