Adenoid cystic carcinoma of Bartholin’s gland is a rare tumor. Its management is inspired by the treatment of vulvar cancers. We present here a clinical case of a 28-year-old female patient who presented with a painful swelling of the left labia majora, which was mistaken for simple bartholinitis and treated medically without improvement. A biopsy was then performed, and the histopathological study revealed a cystic adenoid carcinoma of the Bartholin gland. The radiological workup showed a locally advanced left vulvar process, without distant metastasis. Due to the impossibility of surgery, concomitant radiotherapy and chemotherapy were performed, with good improvement. The rarity of this type of vascular cancer makes its diagnosis late, because it is often confused with bartholin gland cyst and bartholinitis. Moreover, its management is still not standardized.
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