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Adenoid cystic carcinoma (ACC) of the Bartholin's gland is one of the rarest neoplasms of the female genital tract. Including this report there are 65 cases mentioned in the literature. We report a case of a 36-year-old woman who presented at our hospital after excision of the right Bartholin gland elsewhere which proved to be ACC. The therapy of this rare tumor has many controversial questions and dilemmas, especially in young patients. Our patient underwent surgical treatment only (hemivulvectomy and lymph node dissection) without radiotherapy and is free of disease eight years after.

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