Adenoid cystic carcinoma (ACC) of the breast is a rare subtype of invasive breast cancer. In recent years, some progress has been made in the study of ACC of the breast molecular markers, which lays foundation for the prevention and treatment of ACC of the breast. The MYB-NFIB fusion gene is specifically found in adenoid cystic carcinoma, and the MYB-NFIB fusion gene and/or MYB are closely related to the development of ACC of the breast. In addition, overexpression of epidermal growth factor receptor (EGFR) in ACC of the breast suggests that anti-EGFR targeted therapy may have good effect in ACC of the breast. Coexisting mutations in phosphatase and tensin homolog deleted on chromosome ten and phosphoinositide-3-kinase catalytic alpha polypeptide gene may be associated with metastasis of ACC of the breast. Estrogen receptor-α36 is expressed in some cases of ACC of the breast, suggesting that anti-estrogen therapy may be effective in ACC of the breast patients. Part of ACC of the breast overexpresses insulin-like growth factor-Ⅱ mRNA-binding protein 3, and its overexpression intensity may be related to prognostic factors such as metastasis. The expression level of enhancer of zeste homolog 2 (EZH2) in ACC of the breast was positively correlated with Ki-67 and correlated with metastasis. EZH2 may be an important indicator to judge the prognosis of ACC of the breast and guide the scope of surgery. Topoisomerase Ⅱα in ACC of the breast is not expressed or weakly expressed, suggesting that anthracycline chemotherapy appears to be ineffective in ACC of the breast patients.