Nasopharyngeal carcinoma (NPC) is an epithelial tumor with a distinct geographic distribution and characteristic histologic appearance. It is rare in Europe and North America, but it is among the most common cancers in southern China. Genetic predisposition, environmental factors, and Epstein-Barr virus (EBV) all have been associated with the pathogenesis of this tumor. There is an increasing body of evidence that among all these factors, EBV appears to be the strongest and most consistently related factor. According to the current sensitive in situ hybridization methods for the detection of EBV-encoded small RNAs (EBER), almost 100% of cases of NPC, irrespective of their histologic subtypes, have demonstrable EBERs in the nuclei of the tumor cells. In this review paper, we discuss the predisposing genetic and environmental factors and the role of EBV in the pathogenesis of this tumor with particular emphasis on the role of EBV.