The Epstein-Barr Virus-associated T- and NK-cell lymphoproliferative disorders are a group of hematologic disturbances that occur in pediatric age groups. The literature describes extensive heterogeneity in the presentation of this disease, with a noncanonical reaction to Epstein-Barr virus infection underpinning the wide variety of clinical manifestations. In this review, we discuss chronic active Epstein-Barr virus infection, hypersensitivity to mosquito bites, Alice in Wonderland syndrome, Hydroa vacciniforme-like lymphoproliferative disorder, and the clinical and histologic findings of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis and systemic Epstein-Barr-positive T-cell lymphoproliferative disorder of childhood. Several case reports of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis and systemic Epstein-Barr positive T-cell lymphoproliferative disorder of childhood describe extensive hemophagocytic lymphohistiocytosis, suggesting overlap between the various entities of Epstein-Barr Virus-associated T- and NK-cell lymphoproliferative disorders. We hope to provide a comprehensive overview of several possible manifestations of EBV-associated systemic T-cell lymphoproliferative disorders and demonstrate that extensive overlap occurs between the various entities.