Nasopharyngeal carcinoma (NPC) is a tumor of epidermal origin with endemic distribution among well-defined ethnic groups and geographic regions. NPC is rare among European and North American Caucasians, but it is one of the most common cancers in southern China, Hong Kong, and Southeast Asian countries including Taiwan, Singapore, Malaysia, Indonesia and Vietnam. Its close association with Epstein-Barr virus (EBV) makes it a model for viral carcinogenesis in humans. This article will first provide an overview on the virology and biology of EBV infection and discuss the etiologic factors identified for NPC such as EBV, environmental risk factors and genetic factors. The evidence for the role of EBV and its potential interplay with environmental and genetic factors in the carcinogenic process of NPC will be reviewed. The last section will discuss the diagnostic markers, current status of therapy including radio- and chemotherapy and move toward introducing the cytotoxic T-cell targeting immunotherapy and the development of potential therapeutic gene therapy of NPC.