Background: Biliary tract carcinoma is infrequent; usually majority of cases are detected in an advanced phase of the disease, thus surgical resection is not feasible and prognosis is poor, mean survival is 6 months and, chemotherapy is the main therapeutic option. Objective: An overall review of all clinical trials published regarding gemcitabine, alone or in combination, as a treatment in advanced biliary tract carcinoma. Results: Gemcitabine has been reported as a single drug, in 12 trials and as a combination in 21 studies. As a single agent it has been evaluated in a 30 minute infusion, biweekly administration, fixed infusion [10 mg/m2/min] or as a prolonged infusion [24 hours]. Objective response has been reported between 0 and 36%, stable disease 13 to 15%, time to progression 2 - 10.7 months, overall survival 4 to 14 months. Chemotherapy combinations based on gemcitabine have been evaluated with several agents, among them were 5-FU, mitomycin oxaliplatin, capecitabine, cisplatin, docetaxel and irinotecan; the objective response seen: 9.3% to 64%, stable disease 9.3% to 53%, time to progression 3 - 10 months and overall survival 4.7 to 18 months. Conclusion: Gemcitabine is an effective drug in advanced biliary tract carcinoma with a low toxicity profile. It should be considered as the standard treatment for unresectable or metastatic disease while awaiting phase III results.