Background: Non-Hodgkin's lymphoma (NHL) may involve the lower female genital tract, most often as a manifestation of systemic disease and rarely as a primarily localisation.
Case: A 73-year-old woman, HIV-negative, presented with a 5-month history of a mass in the left Bartholin's gland. The performed biopsy was reported to be a poorly differentiated carcinoma. Therefore, the patient underwent a vulvectomy with superficial groin node dissection. Unexpectedly, the definitive histological diagnosis showed that the tumor was an extranodal diffuse large B-cell non-Hodgkin lymphoma.
Conclusion: This is the first report of a NHL located in the Bartholin's gland. Primary NHLs involving the external genitalia are rare and often inaccurately diagnosed. A greater awareness of this entity among clinicians and pathologists could uncover more cases.