Focal strictures occurring at the hepatic duct confluence, or within the common hepatic duct or common bile duct in patients without a history of prior surgery in that region or stone disease, are usually thought to represent cholangiocarcinoma until proved otherwise. However, not uncommonly, patients undergo surgical exploration for a preoperative diagnosis of cholangiocarcinoma, based on the cholangiographic appearance of the lesion, only to find histologically that the stricture was benign in nature. Despite sophisticated radiographic, endoscopic, and histologic studies, it is often impossible before laparotomy to distinguish malignant from benign strictures when they have the characteristic radiographic appearance of cholangiocarcinoma. Even at the risk of overtreating some benign cases, most agree that aggressive surgical resection is the treatment of choice, given the serious consequences resulting from a failure to diagnose and adequately treat cholangiocarcinoma. Four patients who presented to our institution between February 1991 and June 2000 underwent laparotomy for a preoperative diagnosis of biliary tract malignancy based on clinical presentation and cholangiographic findings. The final pathology report in all patients showed marked fibrosis and inflammation of the biliary duct without evidence of malignancy. A review of the patient data and the relevant literature identified benign causes of focal extrahepatic biliary strictures associated with concomitant disease processes in two of the four patients. We present these cases and discuss the benign etiologies with emphasis on the role of surgery in both diagnosis and treatment.