The adenoid cystic carcinoma (ACC) of the Bartholin’s gland (BG) is one of the most uncommon variant of vulvar malignancies representing only 10–15% of cases. The main differential diagnosis is the BG cyst. Risk factors to the development of ACCBG are still unclear. The symptoms are usually non-specific and may include local inflammation, pain, local itching, burning sensations, bleeding, pruritus and or dyspareunia. There is currently no consensus regarding the optimal surgical treatment and the question whether to do or not a systematic inguinal femoral lymph node dissection is still controversial. Guidelines for postoperative chemotherapy or chemoradiotherapy are not established, despite the relative frequency of microscopically positive surgical resection margin. Adjuvant radiation therapy seems to lower the incidence of local recurrence in patients with positive resection margins, based on retrospective studies and case reports. Chemotherapy as adjuvant treatment is still under evaluation