Adenoid cystic carcinoma (ACC) of Bartholin’s gland is an extremely rare tumor of the female genital tract, representing about 5%-15% of Bartholin’s gland malignancies. Approximately 80 cases have been reported in the literature. The authors describe the case of a 54-year-old woman with locally advanced ACC of Bartholin’s gland treated with surgery and adjuvant radiotherapy (RT). She underwent radical hemivulvectomy associated with ipsilateral inguinal and femoral lymph node dissection. Subsequently, she received postoperative three-dimensional conformal RT. Total dose prescribed was 56 Gy in 28 fractions of two Gy each. After 20 months of follow up, there was no evidence of local failure or distant progression.