<p><b>OBJECTIVE</b>To evaluate the clinicopathological characteristics and treatment of adenoid cystic carcinoma of the Bartholin gland.</p><p><b>METHODS</b>The clinicopathological data of six patients with adenoid cystic carcinoma of the Bartholin gland were retrospectively analyzed. The median age was 40.8 years (range 30 to 54 years). Surgery was the primary treatment. Simple vulvar tumor resection was performed in 1 patient. Four cases underwent radical vulvectomy with bilateral inguinal lymph node dissection and 1 case underwent wide local excision of the vulva with bilateral inguinal lymph node biopsy. Two cases with high risk factors received postoperative radiotherapy.</p><p><b>RESULTS</b>All patients had definite pathological diagnosis. Cribriform arrangement of tubules and gland-like elements and infiltration of perineural spaces were two main microscopic features of this type of tumor. The pathological examination after surgery revealed that two patients had positive surgical margins, one had negative margin, 1 adjacent to the tumor and 1 unknown; 5 cases had negative inguinal lymph nodes and 1 unknown. All the 6 patients were followed-up. Recurrence developed in 4 cases including 3 with both local recurrence and lung metastasis, and one had lung metastasis only. One patient died of lung metastasis and her total survival period was 135 months. The other 3 recurrent patients survived with tumor and the total survival period was 241, 128 and 103 months, respectively. Two cases without recurrence survived 8 and 121 months, respectively.</p><p><b>CONCLUSION</b>Adenoid cystic carcinoma of the Bartholin gland is a slow growing but locally very aggressive neoplasm with a high capacity for local recurrence and lung metastasis. Surgery is the most common and useful treatment. Radiation is a choice of treatment for patients with high risk factors after surgery such as positive surgical margin, deep local invasion and infiltration of perineural spaces or for recurrent patients without opportunity of excision.</p>