Abstract: Lacrimal gland adenoid cystic carcinoma (LGACC) is the most common primary malignant epithelial tumor of the lacrimal gland and has the characteristics of high recurrence rate, neurotropic growth, and risk of distant metastasis. The clinical manifestation is mainly a mass in the lacrimal gland area with a poor specificity. The combination of MRI and CT is important in the diagnosis of LGACC, and PET/CT is of great value in determining the nature and metastasis of LGACC. Histopathological examination is the gold standard for diagnosis, and the type of histopathology is significant for predicting the prognosis of patients. At present, the most commonly used treatment for LGACC is surgical resection combined with postoperative local radiotherapy. However, the overall prognosis is poor after active treatment because the pathogenesis of LGACC remains unclear. This article will combine relevant literature and the author's clinical experience to review the epidemiology, clinical manifestations, imaging examination, histopathological changes, treatment, and prognosis of LGACC. Results will contribute to improve the quality of diagnosis and treatment and the prognosis of patients.