BACKGROUND Surgery is the only curative therapy for patients with hilar cholangiocarcinoma (HCCA). Combined portal vein resection (PVR) could achieve negative resection margins in HCCA patients with portal vein invasion. This systematic review aimed to analysis the efficiency of combined PVR for HCCA. METHODS MEDLINE, EMBASE, the Cochrane Library, the Chinese National Knowledge Infrastructure database, and clinical trial registries were searched through April 2015. Risk ratios (RRs), and 95% confidence intervals (CIs) were calculated. RESULTS The analysis included 21 retrospective studies, altogether involving 2403 patients (patients with PVR, n=637; patients without PVR, n=1766). Patients with PVR were likely to have more advanced HCCA (lymphatic invasion: RR=1.14, 95% CI 1.02 to 1.28; perineural invasion: RR=1.31, 95% CI 1.05 to 1.63) and suffered less curative resections (RR=0.89, 95% CI 0.75 to 0.99). Postoperative morbidity was similar between patients with or without PVR (RR=1.06, 95% CI 0.94 to 1.02). Patients with PVR suffered higher mortality rate (RR=1.52, 95% CI 1.06 to 2.18), and worse 5-year survival rate (RR=0.67, 95% CI 0.49 to 0.91). CONCLUSION Combined PVR for HCCA patients would not increase postoperative morbidity rate. However, ascribed to PVR group concluded more advanced HCCA patients; patients with PVR had increased postoperative mortality rate and worse survival rate. The results still need further high quality trails for validation.