Adenoid cystic carcinoma(ACC) of the lacrimal gland is the most common malignant epithelial tumor of the lacrimal gland. It is classified into the crimoid type, tubular type, solid type and high-level transformation. The lacrimal ACC solid type has higher rate of metastasis and recurrence and lower surviral rate than the crimoid type and tubular type. However, high-level transformation of lacrimal ACC has higher rate of metastasis, recurrence and mortality than solid type. In terms of molecular biology, it has been found that MYB, miR-24-3p, bcl-2, P53 and other genes are related to the occurrence of this tumor, while positive expression of MMP-2, MP-9, VEGF, HIF-1α and other genes are related to the prognosis and recurrence of the larcrimal gland ACC. (Int Rev Ophthalmol, 2020, 44: 426-430)