BRAF inhibitors were approved by US Food and Drug Administration as the treatment of patients with unresectable or metastatic melanoma with the BRAF V600E mutation, opening the era of targeted therapy. As acquired resistance to the BRAF inhibitors became problematic, however, combination strategies were suggested. We conducted a systematic review and meta-analysis to assess the efficacy of combination therapy including BRAF inhibitors as treatment of advanced melanoma with V600 BRAF mutation.