Primary cutaneous follicle center lymphoma (PCFCL) is a rare, indolent neoplasm with a good prognosis. Transformations of PCFCL into more aggressive non-Hodgkin lymphomas, such as diffuse large B-cell lymphoma (DLBCL), are extremely uncommon, with few cases reported in the literature. We present a case report of an 84-year-old man with a history of PCFCL who presented 26 months after initial treatment with an enlarged lymph node in the right neck. Excisional biopsy revealed DLBCL. Further studies showed the presence of Epstein-Barr virus (EBV), and molecular studies were consistent with the two neoplasms being clonally related. Taken together, these findings suggest an EBV-driven transformation of PCFCL to DLBCL in this patient.