Abstract: Intraductal papillary mucinous neoplasm of the bile duct is a rare kind of papillary neoplasm of the bile duct. In the recent years, relative reports have been growing in the number quality. The main clinical manifestation is the obstructive jaundice caused by mucinous proteins secreting by the mucinous neoplasms. With the help of computed tomography and magnetic resonance cholangio paneretography, the bile dilation, the bile occupying and filling-defect can be observed. Through the choledochoscope large amounts of mucous-like gall can be seen. And the pathological tissue biopsy can confirme the diagnosis. This disease is mostly benign but at times malignant. Almost limited lesions are treated by radical excision and prognosis is always be fine, while diffuse lesions prefer the chemotherapy but the effect is poor and the prognosis is not good.