Abstract: Intraductal papillary neoplasm of the bile duct (IPNB) is a rare type of biliary tract neoplasms characterized by biliary dilatation, papillary neoplasm or cystic changes, with or without mucous secretion. The etiology and mechanism of IPNB are still unclear. Studies have shown that its pathogenesis is closely related to cholestasis and repeated biliary tract infection caused by cholestasis and clonorchionchus infection and other diseases. Signaling pathways such as Ras‑MAPK and Wnt/β‑catenin play important roles in progression of IPNB. IPNB lacks specific clinical features. Its symptoms depend on the location of tumor and the degree of bile duct obstruction. The main manifestations are abdominal pain, obstructive jaundice, fever, and recurrent cholangitis. IPNB can be divided into pancreatobiliary type, intestinal type, gastric type and eosinophilic type according to epithelial tissue morphology and immunohistochemical staining markers. Based on the location of main lesions, IPNB can be divided into intrahepatic, extrahepatic and diffuse types. The prognosis of patients of IPNB receiving radical surgery is better than those receiving palliative treatment, and all patients with indications are recommended to receive radical surgery. The authors summarize the etiology, progression mechanism, clinical manifestations, pathological features, diagnosis and treat-ment status of IPNB, and provide a review of its research progress.