Although, Nasopharyngeal Carcinoma (NPC) is uncommon in Arab countries, but its incidence is raising due to increased exposure to diverse risk factors. Many of the NPC-related risk factors are becoming more and more apparent in Saudi Arabia. Risk factors such as, higher antibody titers against the EBV, intake of preserved foods, tobacco smoking with alcohol consumption, family history of NPC, certain human leukocyte antigen class I genotypes, history of chronic respiratory tract conditions, exposure to different inhalants, herbal medicines, and occupational exposures are frequently encountered in Saudi population. In spite of the importance of this subject in Saudi Arabia, there is still a paucity of literature on the features and outcome of NPC in the Middle East and most Arab countries. Therefore, the objective of this review was to provide an overview of NPC and recent advances in the multidimensional understanding of this disease focusing in the available literature in epidemiology and risk factors with especial emphasis in Saudi Arabia. The previous literature was retrieved through electronic search in Medline, PubMed, Cochrane, ScienceDirect, and other electronic data base, as shown in the citation.