We report an elderly patient who presented with a single cutaneous nodule of primary cutaneous Hodgkin's disease (PCHD). Reed-Sternberg cells, expressing CD30 and CD15, but not leukocyte common antigen (CD45RB), were identified in the neoplastic cells. Southern blot analysis failed to detect T-cell receptor or immunoglobulin gene rearrangement. A work-up for systemic disease at the time of presentation was negative, supporting the diagnosis of PCHD. Nevertheless, 1 year later, the disease course was complicated by the development of nodal HD and nonHodgkin's lymphoma (NHL). This case report supports the existence of this rare, but distinct, cutaneous tumor.