Dramatic improvements in the outcomes of patients with nasopharyngeal carcinoma (NPC) have prevailed in the past few decades since the advent of megavoltage radiotherapy in the 1960s. The better diagnostic imaging, improvement of radiotherapy (RT) technology, the broader application of chemotherapy, and the more precise disease staging have all contributed to the achievements. However, distant control remains a crucial problem, the prognosis of patients with distant metastases remains disappointing, and reducing late toxicity while preserving the local control continues to be a challenging issue particularly in those demonstrating intracranial extension. The etiological association between Epstein-Barr virus (EBV) and NPC has led to the discovery of the biomarker for prognostication and treatment monitoring, as well as provided an arena for novel drug development. Significant progress in the immune-oncology has also emerged with encouraging preliminary results for this disease. This article will review the key advancements in the management of NPC in the endeavor and highlight the current challenges.