Introduction: Biliary adenomas are rare entities that are usually detected incidentally in gallbladders removed for unrelated indication.They can also occur in the extrahepatic biliary tree, but true incidence is not known.Currently, there is limited understanding of the malignant potential of biliary adenomas involving the extrahepatic tree, and there are no guidelines for management. The aim of our study is to analyze a group of patients with extrahepatic biliary tree adenomas and study their clinical course. Methods: The pathology database at Baystate Medical Center was used to identify patients diagnosed with biliary adenomas from the year 2000 to 2013. Patients’ medical records were reviewed for demographics and clinical course. Results: Pathology results from 8,774 cholecystectomies (with or without bile duct excision) and 1,785 bile duct biopsies over a 13-year period were reviewed.Twenty-three patients with biliary adenoma were identified, arising in the gallbladder (20/23) or the extrahepatic biliary tree (3/23). All 3 patients with extrahepatic biliary adenoma were female, with a mean age of 74 years. On initial presentation, none of the patients had obstructive jaundice. Case 1 had biliary dilation seen on imaging, and endoscopic ultrasound revealed a adenomatous polyp in the distal bile duct. The patient refused surgery and presented with occlusive biliary stricture and jaundice 5 months later. Cytology confirmed malignant progression. Case 2 had history of primary sclerosing cholangitis and presented with cholangitis, gram negative sepsis. A polypoid lesion was seen on imaging in the common hepatic duct, and direct cholangioscopy showed adenoma with high-grade dysplasia. The patient underwent successful total bile duct resection and hepaticojejunostomy with good outcome. Case 3 presented with symptoms suggestive of gallbladder pathology and was found to have polypoid distal bile duct lesion on intraoperative cholangiogram. Endoscopic retrograde cholangioscopy showed adenomatous polyp with high-grade dysplasia. The patient underwent distal bile duct resection with choledochojejunostomy, but presented with jaundice 4 years later secondary to adenocarcinoma involving small bowel in the Roux limb of jejunum and transverse colon. Conclusion: Adenomas involving the extrahepatic biliary tree are rare benign tumors of the biliary tract. These patients tend to have aggressive clinical course with high risk of subsequent malignancy. Given the poor prognosis associated with cholangiocarcinoma, we suggest aggressive management with total bile duct resection along with hepaticojejunostomy if biliary adenoma is diagnosed outside the gallbladder. Close surveillance for other gastrointestinal malignancies might be warranted. Further analysis of larger databases is recommended.