Epstein-Barr virus (EBV)–associated lymphoproliferations involving the skin are a rare but important group of diseases with a broad spectrum of behavior, ranging from self-limiting spontaneously resolving disorders to highly aggressive malignancies. They may be of B, T, or natural killer (NK) cell type and include EBV-positive mucocutaneous ulcer, lymphomatoid granulomatosis, EBV-positive diffuse large B-cell lymphoma, hydroa vacciniforme–like lymphoproliferative disorder, and extranodal NK/T-cell lymphoma of nasal type. Recognition and distinction of these entities is important in view of their differing prognoses and treatments. An association with EBV may be the first indication that a patient is immunosuppressed.