Background: Anti-programmed death receptor-1 (PD-1) agents improve survival in patients with recurrent or metastatic nasopharyngeal carcinoma (NPC), but have not been studied well in patients with locoregionally advanced NPC. In this study, we investigated the efficacy and safety of the PD-1 inhibitor camrelizumab combined with gemcitabine and cisplatin as neoadjuvant therapy in patients with locoregionally advanced NPC. Methods: This was a single-arm, single-center, phase II trial done at the First Affiliated Hospital of University of Science and Technology of China. Patients with previously untreated, stage III-IVB (except T3N0-1; AJCC 8th) NPC, aged 18-70 years without severe comorbidities were enrolled. Eligible patients received neoadjuvant therapy with camrelizumab 200 mg on day 1, cisplatin 80 mg/m² on day 1, and gemcitabine 1,000 mg/m² on days 1 and 8 of each 21-day cycle for three cycles. The primary endpoints were overall response rate(ORR) per RECIST version 1.1 and safety. Biomarker analysis included programmed death-ligand 1 (PD-L1) expression using the tumor proportion score (TPS) and combined positive score (CPS; Dako 22C3 pharmDx assay). Results: Between January 2022 and January 2023, 14 patients were enrolled and treated. The median age was 55 years (range, 33-68), and 11 (78.6%) patients were males. Thirteen (92.9%) patients had baseline PD-L1 data: TPS ranged from 2% to 80% and CPS ranged from 5 to 90. Eight patients (61.5%) had PD-L1 TPS of higher than 20% and seven patients (53.8%) had PD-L1 CPS of higher than 50. The ORR was 92.9% (13 of 14), including one (7.14%) patient with complete response and 12 (85.7%) patients with partial response. The most common grade 3 treatment-related adverse events (TRAEs; Common Terminology Criteria for Adverse Events, version 5.0) during neoadjuvant treatment were anorexia (two of 14;16.7%) and nausea (two, 9.5%), and no grade 4 or 5 TRAEs occurred. The most common immune-related AEs were endocrinopathies (all grade 1; 50%). Conclusions: Neoadjuvant camrelizumab plus chemotherapy showed promising activity with a manageable safety profile for patients with locoregionally advanced NPC. Longer follow-up and larger studies are needed to further confirm the efficacy of this combination in this population. This trial was approved by the Ethics Office of the First Affiliated Hospital of the University of Science and Technology of China (2021 no98), and registered in the Chinese Clinical Trial Registry. Clinical trial information: ChiCTR2200057745.