CD56+ hematological neoplasms presenting in the skin have a poor prognosis, except for primary cutaneous CD30+ lymphoproliferations. The striking similarities between blastic NK-cell lymphomas and CD56+ myeloid leukemias presenting in the skin provide a rationale to treat these patients with more aggressive regimens, rather than with CHOP(-like) regimens and radiotherapy, which have proven to be inadequate therapies for this neoplasm.