Neuroma of the biliary tract is a rare condition thought to be caused by trauma secondary to cholecystectomy. More rare is the occurrence that causes symptomatic biliary obstruction. A 65-year-old woman was hospitalized because of abdominal pain, nausea, vomiting, and general malaise of 1 to 2 months duration. Cholecystectomy had been performed 40 years before. Ultrasound revealed hepatomegaly and dilated intrahepatic ducts. CT showed intra- and extrahepatic ductal dilatation with questionable intraductal mass. Endoscopic retrograde cholangiopancreatography and percutaneous transhepatic cholangiography demonstrated stricture of the hepatic duct bifurcation. The biliary bifurcation was resected, and hepaticojejunostomy was performed. The patient's postoperative course was unremarkable. Histological examination of the surgical specimen revealed positive staining for the S-100 antigen of the obstructing luminal stricture (without evidence of cholangiocarcinoma), which was consistent with a biliary neuroma. Positive staining was also found for acidic (and not basic) fibroblast growth factor (FGF) and two of its high affinity receptors (FGFR-1 and FGFR-4). This study supports the apparent association between biliary neuromas and cholecystectomy as well as the potential role of an established angiogenic and neurogenic growth factor in the formation of this tumor. Finally, this case is also unique in that it represents the longest interval between cholecystectomy and presentation of a biliary neuroma, 40 years after surgery.