Bartholin Gland (BG) carcinoma is an uncommon malignancy, comprising less than 5% of all vulvar cancers [1]. Adenoid Cystic Carcinoma (ACC) makes up 10-30% of these cases, following squamous cell carcinoma and adenocarcinoma in prevalence [2]. While ACC is most frequently observed in the minor and major salivary glands, it can also manifest in other secretory glands, such as the tracheobronchial tree, esophagus, breast, and BG. ACC is marked by a slow progression and low tendency for lymph node involvement, but it has a significant potential for hematogenous metastasis, which can occur long after the primary tumor is diagnosed [3]. Because of its rarity, there are no specific recommendations for the management of ACC of BG (ACCBG) and the treatment must be decided in multidisciplinary staff and following the guidelines of the vulvar cancer.