Introduction: A primary cutaneous CD-30 positive T -cell lymphoproliferative disorders are rare and heterogeneous group of primary skin tumors, which include primary cutaneous anaplastic large cell lymphoma (PCALCL) and lymphomatoid papulois.
Case Report: We report a rare an atypical case of PCALCL with an aggressive and refractory behavior that occurred in the lip vermilion, and that had been initially diagnosed as aggressive herpes. The lesion was recurrent and refractory to the CHOEP chemotherapy protocol followed by radiotherapy, but after hyper CVAD (acronym) + brentuximab with a considerable improvement.