Cutaneous lymphomas are a heterogenous group of lymphoproliferative disorders of the T- and B-lymphocytes with a low incidence of approximately 1:100000/year. They belong to the Non-Hodgkin lymphoma. The skin is the second most abundant site of extranodal lymphoma formation (after the GI tract). The new WHO/EORTC classification of cutaneous T- and B-cell lymphomas provides a widely accepted nomenclature for primary cutaneous lymphomas based primarily on clinical, but also on histologic, cytologic and molecular features. It has already proven to be an invaluable tool for international prospective clinical studies. The clear distinction of primary cutaneous from secondary cutaneous lymphoma will also be important to prevent overtreatment of the frequently benign primary cutaneous lymphoma. Treatment of primary cutaneous lymphoma is skin-directed in early disease stages, and uses as systemic approach in advanced stages. Skin-directed therapies encompass UV-light treatment such as UVB311nm, or PUVA, topical steroids class III and IV, or bexaroten gel. Systemic treatment options may be immunomodulatory, such as treatment with interferon alpha injection, or biologic response modifiers such as bexarotene. We recommend that advanced stages of cutaneous lymphoma should be treated in centers that offer clinical studies in this field, because prognosis of late stages is still dismal and there is so far no therapeutic approach that has led to an increase in overall survival. Hence, inclusion of patients in prospective controlled clinical studies should always be considered in patients with primary cutaneous lymphoma.