Adenoid cystic carcinoma (ACC) of the Bartholin's gland (BACC) is an extremely rare malignancy of the vulva. The characteristics of BACC include slow tumor growth and aggressive invasion, especially with perineural involvement. A 64-year-old Japanese woman complained of a mass and pain in the perineum. A 3 cm-sized mass was palpated inside of the labia majora. Diagnostic imaging demonstrated the tumor invading the anus and urethra. The patient underwent total pelvic exenteration with radical vulvectomy. Pathological findings revealed that the tumor was BACC. The surgical margin was positive for tumor cells, which necessitated adjuvant radiotherapy. No serious complications were associated with the surgery and radiotherapy. There is no clear consensus on to the proper surgical intervention in BACC. Achieving tumor-free surgical margins is desired to avoid recurrence. However, such attempts have often failed in reported BACC cases with extended surgical resection. Nevertheless, given the aggressive nature of BACC, extensive surgery including total pelvic exenteration is justified, particularly with promising reported efficacy of adjuvant radiotherapy on BACC prognosis.