A case of mycosis fungoides showing an acute course is presented. A 63-year-old man complained of an erythematous skin lesion with pruritus in May 1995. It was treated with topical steroids and an anti-allergic agent, but the condition repeatedly improved and worsened. The skin erythema became generalized and tumorous on the head and lower extremities. Slight lymph node swelling was observed, but there was no hepato-splenomegaly. Skin biopsy revealed diffuse infiltration of lymphoblastoid cells in the subdermis. Infiltrating lymphocytes showed a T-cell phenotype on immunohistological staining, and Southern blotting analysis revealed rearrangement of the T-cell β chain gene. Mycosis fungoides was diagnosed and the patient was treated with several coursesof combination chemotherapies. Theareafter, the skin lesions improved substantially. However, these including the ulcerative lymphoma of the oral cavity aggravated, which became refractory to treatment by combination chemotherapy. Local electron beam radiation and α-interferon were administered, but the patient died in August 1996.