This study is a case report and literature review of the surgical approach to papillary lesions of the biliary tract exclusive of the ampulla of Vater. Papillary lesions of the bile ducts, exclusive of the ampulla of Vater, are distinctly uncommon but, because of their unpredictable and aggressive behavior, pose challenging problems for the surgeon. Including the present illustrative case description, an English language literature review was conducted to determine the number and clinical behavior of papillary lesions of the bile ducts, particularly the propensity for malignant transformation, and the most favorable surgical approach. In addition to the present case, 29 patients with papillary biliary lesions were found in the literature. Twenty-two patients had tumors in multiple locations in the biliary tract, 6 had isolated lesions in one hepatic duct, and 3 had apillary neoplasia of the bile ducts is a rare P disorder affecting the lining epithelium. The condition includes a spectrum of pathological findings ranging from solitary benign tumors to multiple tumors to diffuse papillomatosis. Although often histologically benign, these lesions are characterized by a propensity for recurrence, mucin production, and malignant transformation. Indeed, benign tumors of the bile ducts exclusive of the ampulla of Vater of any type are distinctly uncommon. Marshall' in 1932 reported the 20-year experience at the Mayo Clinic with bile duct tumors. In over 20,000 operations on the biliary tract, 49 cases of carcinoma were found, but only 4 cases of benign tumors were encountered, none of which were papillary. Rogers' in 1946 described a patient with a recurrent "papillary adenoma" of the common hepatic duct with progressive extension into the left hepatic duct producing cholangitis, liver abscesses, and eventual death. In Chu's3 1950 report on a collected series of 30 well-documented tumors of the extrahepatic From the Departments of Surgery and Pathology, University of Address reprint requests to Thomas 5. Helling, MD, 4320 WomafI