The clinical characteristics and the microscopic and immunohistochemical characteristics of a case with adenoid cystic carcinoma of Bartholin’s gland were analyzed with a follow-up period. Related literature was reviewed.The case was found with a right vulva pain,and was clinically diagnosed as Bartholin’s gland abscess. But under the light microscope, the following can be seen in the patient’s removed perineal mass. The tumor cells are small and uniform, arranged in a basal cell-like, adenoid or atypical sieving pattern. The secreted mucus or red-stained homogeneous cylindrical body can be seen in these glands or meshes of different sizes. In addition, scattered tumor cells are also found in nerve tissues and peripheral muscle tissues. Its immunohistochemical markers are glandular epithelial and myoepithelial positivity. Adenoid cystic carcinoma of Bartholin’s gland is a very rare malignant tumor that occurs in female vulva. Therefore, in addition to clinical diagnosis, pathological biopsy and immunohistochemical detection are required to confirm the diagnosis.