Nasopharyngeal carcinoma (NPC) has a striking geographical and ethnical distribution. It occurs with high frequency in southern China and Southeast Asia. Family clustering was also observed in NPC and a typical family with 15 NPC cases was introduced in this paper. Epidemiological and genetic studies have been carried out in the previous decades and vast information was accumulated for familiar NPC, in terms of risk factors, inheritance mode, and involvement of gene polymorphisms. The major findings in this field were summarized. Furthermore, future directions leading to understanding the genetic mechanism of the familial form of NPC was also discussed.