Malignant lymphoma is a rare entity in the neoplasms of the female genital tract, and thus a case of malignant lymphoma of the vulva is reported in this paper. On admission, the patient, a 37-year-old, Japanese female, had complained of vulvar swelling and pain that had persisted for one month. Stage IV tumors were noted in the vulva and bladder and a histological diagnosis indicated non-Hodgkin's lymphoma with diffuse, large cell and B cell types, according to the classification of the Lymphoma Study Group. The patient thus was treated with CH* OP therapy, which consisted of Cyclophosphamide, Aclarubicin Vincristine (Oncovin), and Prednine , and given brain irradiation. Unfortunately her condition has rapidly deteriorated during the 5 months since her admission.