Malignant lymphomas of the female genital tract are rare. When it involves the female genital tract, information in the literature regarding the classification, treatment and diagnosis of malignant lymphoma are few. Non-Hodgkin lymphomas (NHL) of the female genital tract occurs primarily in the ovary (49%), uterus (11%), vagina (7%), and vulva (4%). The vulva as the primary location is a very rare occurrence. However, autopsy studies have shown that about 40% of women who die of non-Hodgkin's lymphoma had involvement of the uterus or ovaries. There have been many cases of high-stage lymphomas presenting as secondary involvement of the female genital tract, but when the primary involvement site is the vulva, the stage of lymphoma was most commonly stage IE or IIE. Therefore, physicians should keep in mind the possibility of the presence of a malignant neoplasm when the suspected Bartholin's gland tumor does not respond to treatment. The authors propose that thorough gynecological evaluation should be conducted as a routine screening method in female patients with suspected lymphoma. We represent a case of primary non-Hodgkin's lymphoma patient who was relatively young in age and who was consequently determined to be of a high stage.