Nivolumab and ipilimumab (CTLA-4 inhibitor) as a combinatorial therapy are being evaluated in rare tumors that include NPC (ClinicalTrials.gov Identifier: NCT02834013), and nivolumab with chemoradiation in advanced-stage NPC is being evaluated in a phase 2 clinical trial (ClinicalTrials.gov Identifier: NCT03267498) and in a randomized phase 3 trial of adjuvant PD-1 antibody or observation in patients with locoregional advanced NPC (ClinicalTrials.gov Identifier: NCT03427827). This evidence concerns the gene CTLA4 and nasopharyngeal carcinoma.