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肝门部胆管癌恶性程度高、发病隐匿，预后不良。根治性手术为首选治疗方法，但根治率低，因此早期诊断尤为重要。目前诊断主要依靠影像学技术如MRI、磁共振胰胆管造影（ MRCP ）等及内镜下逆行胰胆管造影术（ ERCP ）、超声内镜（ EUS）、组织学检查和肿瘤标志物。随着新发现的肿瘤标志物灵敏度、特异度的提高，探索新的肿瘤标志物为肝门部胆管癌早期诊断提供新思路。“,”The hilar cholangiocarcinoma with high malignant degree and insidious development, is associated with poor prog?nosis. 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.