Background: Adenoid cystic carcinoma (ACC) of the Bartholin's gland is a rare malignancy of the female genital tract and there have been 62 cases of ACC of the Bartholin's gland in the literature.
Cases: Two cases of ACC of the Bartholin's gland are reported.
Conclusion: There is no consensus on optimal treatment of ACC of the Bartholin's gland. Most commonly, wide local excision and radical vulvectomy with or without lymph node dissection, are performed. More long-term follow up is recommended to evaluate optimal primary treatment and roles of radiotherapy and chemotherapy because ACC of the Bartholin's gland recurs and metastasizes long after primary treatment.