Epstein-Barr virus (EBV), one of the eight known human herpesviruses, generally infects subclinically. However, in certain circumstances, EBV causes overt diseases such as infectious mononucleosis (IM), usually a selflimiting disorder, by its primary infection. Additionally, it associates the development of various human malignancies including EBV genome-positive Burkitts lymphoma (BL) and undifferentiated nasopharyngeal carcinoma (NPC). More recently, EBV infection has been thought to be etiologically linked to the occurrence of lymphoproliferative disease (LPD) in immunologically compromised individuals either with hereditary or secondary condition, and the other lifethreatening diseases such as hemophagocytic lymphohistiocytosis (HLH) and chronic active EBV infection (CAEBV). This review mainly introduces, focuses and discusses on the recent advances of EBV infection regarding its pathogenetic mechanism(s), diagnosis and treatment.