Mycosis fungoides and Sézary syndrome are low-grade, non-Hodgkin's lymphomas with initial cutaneous involvement. Early stage patients have a good prognosis and are commonly treated with topical agents. However, most patients eventually develop resistant skin disease or visceral disease, necessitating systemic treatment. Single agent and combination chemotherapy have resulted in high response rates, although these are short lived and not without treatment toxicities. Other modalities that have been tried with promising early results are: interferon, fludarabine, retinoids, cyclosporin, 2'-deoxycoformycin, leukapheresis, photopheresis and monoclonal antibodies (unlabeled, radiolabeled, or toxin labeled). We will discuss the available treatment options, as well as our approach to the patient with mycosis fungoides or the Sézary syndrome.