Objective: To study the clinicopathologic features of intraductal papillary neoplasm of the bile duct(IPNB) and to analyze the diagnostic and therapeutic patterns. Methods: The data of 46 patients with IPNB undergoing surgery in Department of Hepatobiliary and Pancreatic Surgery, the Second Affiliated Hospital of Zhejiang University School of Medicine from January 2013 to November 2017 were retrospectively analyzed.There were 23 males and 23 females with age of (64±8)years.Patients were followed up by clinics and telephone inquiry.Categorical data were compared with χ² test or Fisher′s exact test. Results: Abdominal pain(in 31 patients), fever (in 15 patients) and jaundice (in 11 patients) were the most common symptoms.Twenty-five patients were accompanied with cholangiolithiasis and 25 were accompanied with liver atrophy.Preoperative laboratory examination was mainly manifested as the abnormal liver function caused by biliary obstruction.Typical imaging findings included bile duct dilation (in 45 patients) and mass within bile duct (in 22 patients). All the patients were diagnosed as IPNB histopathologically.Among them, high-grade intraepithelial neoplasia and related adenocarcinoma were more common in mucus-hypersecretion IPNB ((13/15 vs. 51.6%(16/31))(χ²=5.331, P=0.021). Hepatectomy was performed in 25 patients, hepatectomy combined with biliary resection and reconstruction in 12 cases, biliary resection and reconstruction in 3 cases, pancreatoduodenectomy in 3 cases, hepatopancreaticoduodenectomy in 1 case, liver transplantation in 1 case and radiofrequency ablation in 1 case.Forty-one patients were followed up with a median of 30 (12, 41) months.Seven patients suffered recurrence and 6 died. Conclusion IPNB is a rare disease with limited knowledge currently.Images are the main diagnositc means and surgery is the first choice.