Intrabiliary papillary neoplasm (IPN-B) is a rare mucus-secreting tumor of the biliary system, mostly found in the intrahepatic bile ducts, and more rarely in the extrahepatic bile ducts. Because IPN-B often secretes mucus to obstruct the bile ducts, it often presents with symptoms such as right upper abdominal pain, cholangitis and obstructive jaundice. In the past, researchers believed that IPN-B represented a spectrum of disease that included a range of conditions from benign to malignant (chronic inflammation, atypical hyperplasia, carcinoma in situ, and invasive carcinoma). IPN-B is often associated with intrahepatic bile duct stones, hepatic schistosomiasis, and chronic proliferative cholangitis, so the latter is often thought to be associated with the development of the former. IPN-B with mucus secretion or intrahepatic bile duct stones can lead to bile duct obstruction, so it often presents with symptoms such as pain in the right quadrant of the rib cage, cholangitis, and obstructive jaundice. However, conventional imaging has a detection rate of less than 50% for intraductal papilloma and mucin secretion, so further determination of the extent and histologic features of the lesion by biliary endoscopy is sometimes required, which is crucial for selecting the appropriate treatment. IPN-B is now considered to be a precancerous lesion with malignant potential and has multiple subtypes. Although the type of IPN-B tumor cells may influence the type of tumor growth and patient prognosis, surgical resection remains the best treatment modality. After complete removal of the tumor, patients can achieve a longer survival time. If surgery is not indicated, adjuvant or conservative treatment is often used to prolong patient survival, such as intra-biliary radiotherapy and bile drainage. IPN-B is a rare bile duct tumor with unique clinicopathological features, but can survive for a long time after complete surgical resection. Therefore, the possibility of IPN-B should be considered when cystic tumors of the liver are encountered in clinical practice for timely diagnosis and selection of appropriate treatment modalities.