Abstract: The hilar cholangiocarcinoma with high malignant degree and insidious development， is associated with poor prognosis. Radical operation is the preferred treatment strategy but the cure rate is low. Thus it is very important to make early diagnosis. This paper summarizes the current diagnosis methods which relied mainly on imaging technology such as MRI and magnetic resonance cholangiopancreatography（MRCP）， endoscopic retrograde cholangiopancreatography（ERCP）， endoscopic ultrasonography（EUS）， histological examination and tumor markers. With the improvement of sensitivity and specificity of the newly discovered tumor markers， it is the new method of early diagnosis of hilar cholangiocarcinoma to discover new tumor markers.