Although platinum-based systemic treatment is the first choice for patients with metastatic nasopharyngeal carcinoma; the best treatment option is still under investigation and it remains a clinical challenging issue. Combination of cisplatin and gemcitabine is proved to be an effective regimen in recurrent/metastatic nasopharyngeal carcinoma. Besides, immuno-drug therapy (anti-programmed cell death ligand 1; pembrolizumab or nivolumab) also displayed a considerable treatment breakthrough for patients with recurrent/metastatic head and neck cancer. However, it is unknown whether combination of immuno-drug with chemotherapy will increase adverse effects or not in patients with nasopharyngeal carcinoma. We shared our clinical experience of therapeutic compliance and acute hematologic adverse effects for patients with metastatic nasopharyngeal carcinoma treating by nivolumab in combination with cisplatin and gemcitabine. Secondary, the clinical tumor response after treatment was also reported. The most common acute hematologic adverse effects were grade 3 anemia followed by grade 2 leukopenia; nevertheless, the patient received this chemotherapy regimen for 12 cycles without interruption. A complete response was documented by [18F]-fluorodeoxyglucose positron emission tomography scan and the patient is still under regular follow up with disease-free status for more than 22 months. In conclusion, combination of nivolumab with cisplatin and gemcitabine does not increase the treatment-related toxicities in patients with metastatic nasopharyngeal carcinoma and this chemotherapy regimen is an effective treatment option.