Adenoid cystic carcinoma of Bartholin’s gland (BG-ACC) is a rare vulvar carcinoma which has its own unique characteristics, such as growing slow, local invasion, perineural infiltration and high local recurrence rates. Signs and symptoms are nonspecific. Perineural invasion may be the reason for causing pain and high recurrence rates. There is no consensus on treatments. We collect all current literatures about BG-ACC to summarize the clinical features, histological features and treatment options. Surgery is the cornerstone of treatments. Adjuvant chemotherapy and radiation therapy may be beneficial in case of distant metastasis. We also need more research in molecular targeting therapy.