ABSTRACT: BACKGROUND:The incidence of a positive microscopic ductal margin (R1) after surgical resection for perihilar cholangiocarcinoma (pCCA) remains high, but the beneficial of additional resection has not been confirmed by any meta-analysis and randomized clinical trials (RCT), which also increased the risk of morbidity and mortality. Hence, a systematic review is warranted to evaluate the clinical value of additional resection of intraoperative R1 for pCCA. METHODS:Eligible studies were searched by PubMed, MedLine, Embase, the Cochrane Library, Web of Science, from Jan.1st 2000 to Nov.30th 2019, evaluating the 1-, 3-, and 5-year overall survival (OS) rates of additional resection of intraoperative pathologic R1 for pCCA. Odds ratio (OR) with 95% confidence interval (CI) was used to determine the effect size by a randomized-effect model. RESULTS:Eight studies were enrolled in this meta-analysis, including 179 patients in the secondary R0 group, 843 patients in the primary R0 group and 253 patients in the R1 group. The pooled OR for the 1-, 3-, and 5-year OS rate between secondary R0 group and primary R0 group were 1.03(95%CI 0.64~1.67, P = 0.90), 0.92(95%CI 0.52~1.64, P = 0.78), and 0.83(95%CI 0.37~1.84, P = 0.65), respectively. The pooled OR for the 1-, 3-, and 5-year OS rate between secondary R0 group and R1 group were 2.14(95%CI 1.31~3.50, P = 0.002), 2.58(95%CI 1.28~5.21, P = 0.008), and 3.54(95%CI 1.67~7.50, P = 0.001), respectively. However, subgroup analysis of the West showed that the pooled OR for the 1-, and 3-year OS rate between secondary R0 group and R1 group were 2.05(95%CI 0.95~4.41, P = 0.07), 1.91(95%CI 0.96~3.81, P = 0.07), respectively. CONCLUSION:With the current data, additional resection should be recommended in selected patients with intraoperative R1, but the conclusion is needed further validation.