Abstract: Nasopharyngeal carcinoma (NPC) is a squamous cell carcinoma arising from the nasopharyngeal epithelium, which is associated with Epstein-Barr virus (EBV) infection and is most common in southern China. Immunotherapy, particularly immune checkpoint inhibitors (ICIs), provides favorable efficacy with a good safety profile in a variety of solid tumors, including recurrent or metastatic NPC (R/M-NPC). In addition, immunotherapy has made new breakthroughs in the treatment of locally advanced NPC, and many clinical studies are underway. Based on previously reported results, this study presents a comprehensive overview of the advances and challenges by introducing the basic principles and the current status of immunotherapy in NPC, with the expectation that it will serve as a reference for clinical practice in the future.