The clinical experience with chemotherapy in mycosis fungoides is practically limited to the action of different types of chemotherapeutic agents on the lesions in far-advanced cases. A large number of agents from almost all classes – alkylating agents, antimetabolites, antibiotics, alkaloids, hormones – have some efficacy in clearing skin lesions. Despite this fact, combination chemotherapy has been the subject of a very small number of publications with conflicting results. The influence on survival, the comparative effects of combination versus single-agent chemotherapy, the value of chemotherapy combined to other treatment modalities have not been submitted to scientific evaluation. Considering the low frequency of the disease and the great variability of the clinical picture, it seems mandatory to implement large cooperative trials, possibly on an international scale.