Adenoid cystic carcinoma (ACC) of the Bartholi`s gland is a rare histological type malignancy of the external genital tract. Only less than eighty cases have been reported in the literature. A 58-year-old women presented to our clinic with chronic external genital pain. In gynecologic examination, slightly hard and swelled mass was palpated around Bartholin`s gland. Under general anesthesia, we performed left hemivulvectomy, inguinal lymphadnectomy, external iliac lymph node sampling by laparoscopy. Pathologic examination conformed ACC showing positive resection margin without lymph node invasion. Postoperative radiotherapy was performed. We planned long-term follow up to evaluate optimal primary treatment and role of radiotherapy.