Abstract: Objective To observe the efficacy and safety of endostar combined with gemcitabine and oxaliplatin as the firstline treatment for patients with advanced biliary tract carcinoma. Methods Forty-eight patients from Jan. 2009 to Aug. 2013 confirmed with pathologic and imaging examination as stage ⅣB primary biliary tract carcinoma were reviewed. Twenty cases received endostar+GEMOX regimen and 28 cases were applied with GEMOX regimen alone. GEMOX regimen was given as follow： gemcitabine 1000mg／m2 iv， d1，d8； oxaliplatin 100mg／m2 iv， d2， 21 days was a cycle. Endostar was given 15mg iv d1-d14, 21 days was a cycle. The efficacy was evaluated strictly after 2 cycles according to RECIST 1.1 criteria， quality of life（QoL）was evaluated accoding to karnofsky scores，safety was evaluated after 1 cycle according to NCI CTC 3.0 version criteria. The time to progress（TTP）and overall survival（OS） were also observed. Results In GEMOX+endostar group， 1 was in CR，3 in PR，12 in SD, and 4 in PD; the response rate（RR）was 20.0％， disease control rate（DCR）was 80.0％; median TTP was 8.6 months and the median OS was 14.0 months; the QoL improved and stable rate was 80.0％. In GEMOX group， 1 was in CR，5 in PR，15 in SD, and 7 in PD; RR was 21.5％， and DCR was 75.0％; the median TTP was 6.0 months and the median OS was 10.0 months; the QoL improved and stable rate was 71.4％. There was statistical difference in TTP and OS between the two groups（P＜0.05）. The most common toxicity in the two groups were myelosuppression， other main toxicities included nause/vommiting, liver dysfaction, peripheral nearitis, skin allergy reaction and etc, mainly in grade 1-2, and there were no sugnificant differences between the two groups（P＞0.05）. In GEMOX+endostar group， only 2 case of non-specific T wave changed. One case was of auricular flutter， and 1 case with mild hypertension. Conclusion GEMOX+endostar as the first-line treatment for advanced BTCs has good efficacy， may improve or stabilize the patients QoL and prolong survival， and the toxicities are well-tolerated， which worth clinical use and further observation.