Recently, rare CD4+/CD56+ hematodermic neoplasm has been described as a distinct clinico-pathologic entity, with aggressive course and poor outcome. Skin is typically involved at presentation, but widespread dissemination to bone marrow is rapid. To date, no standardized therapeutic approach to this disease has been established. As its diffusion mainly concerns elderly patients, only a few paediatric cases have been documented. We report an additional case of CD4+/CD56+ hematodermic tumour that showed a good response to chemotherapy based on a lymphoma protocol. Moreover, we try to analyse features and outcome of a few other paediatric CD4+/CD56+ hematodermic tumours as they are reported in the literature.