Sources of immune deficiency and dysregulation (IDD) are being increasingly recognized and defined, as are IDD-related B-cell lymphoproliferative lesions and lymphomas occurring in these patients. In this review, basic biology of Epstein-Barr virus (EBV) as it relates to classification of EBV-positive B-cell lymphoproliferative disorders (LPDs) is reviewed. Also discussed is the new paradigm of classification of IDD-related LPDs adopted by the fifth edition World Health Organization classification. IDD-related EBV-positive B-cell hyperplasias, LPDs, and lymphomas are discussed with particular attention to unifying and unique features that assist with recognition of these IDD-related lesions and their classification scheme.