Neoplasms of the biliary tract tree are uncommon and have a poor overall prognosis. Although numerous risk factors have been identified, little is known about the pathogenesis of these tumors, and no effective screening technique is available for early detection. Surgery remains the principal treatment modality and the only potential cure, with laparoscopy playing an increasingly important role in determining the resectability of these tumors. The role of postoperative adjuvant chemotherapy and radiation remains controversial. The efficacy of chemotherapy for advanced disease is relatively limited, with response rates of less than 10% for single-agent fluorouracil. Of the newer agents, gemcitabine (Gemzar) holds the most promise in the treatment of these difficult malignancies.