To gain a better understanding of biliary tract intraductal papillary mucinous neoplasm (BT-IPMN).From January 2000 to December 2013, 19 cases of BT-IPMN were retrospectively identified from a total of 343 biliary tract tumors resected in our single institution. Demographic characteristics, clinical data, pathology, surgical strategies, and long-term follow-up were analyzed.The mean age of the 19 BT-IPMN cases was 53.8 years (range: 25-74 years). The most common symptom was abdominal pain (15/19; 78.9%), followed by jaundice (7/19; 36.8%). Cholangitis was associated with most (16/19; 84.2%) of the BT-IPMN cases. Macroscopically visible mucin was detected in all 19 patients, based on original surgical reports. The most common abnormal preoperative imaging findings for BT-IPMN were bile duct dilation (19/19; 100%) and intraluminal masses (10/19; 52.6%). Thirteen (68.4%) cases involved the intrahepatic bile duct and hilum. We performed left hepatectomy in 11/19 (57.9%), right hepatectomy in 2/19 (10.5%), bile duct resection in 4/19 (21.1%), and pancreatoduodenectomy in 1/19 (5.3%) patients. One (5.3%) patient was biopsied and received a choledochojejunostomy because of multiple tumors involving the right extrahepatic and left intrahepatic bile ducts. Histology showed malignancy in 10/19 (52.6%) patients. The overall median survival was 68 mo. The benign cases showed a non-significant trend towards improved survival compared to malignant cases (68 mo vs 48 mo, P = 0.347). The patient without tumor resection died of liver failure 22 mo after palliative surgery.BT-IPMN is a rare biliary entity. Complete resection of the tumor is associated with good survival, even in patients with malignant disease.