BACKGROUND: Adenoid cystic carcinoma (ACC) of the Bartholin's gland is very rare. There is no agreed consensus on the optimal treatment. CASE: In the present study we report the case of a 41-year-old Chinese woman who suffered from ACC of the Bartholin's gland with lung metastases and repeated local recurrence, and discuss the most suitable treatment for late stage ACC of the Bartholin's gland. CONCLUSION: Our experience from this patient suggests that initial conservative surgery of a local lesion, even for later stage patients with ACC of the Bartholin's gland, could be considered.