Adenoid cystic carcinoma of the lacrimal gland (LGACC) is the most common type of malignant epithelial tumor of the lacrimal gland, which is characterized by a high recurrence rate, perineural invasion, and a propensity to metastasize to distant sites. Due to its unclear pathogenesis, LGACC has a poor prognosis and a high mortality rate. In recent years, a range of radiotherapy and chemotherapy have been clinically applied, leading to a shift in the treatment approach for LGACC. This article discussed the advances being made in the treatment of LGACC and provides readers with an overview of the impact of LGACC treatment modalities on patient survival and prognostic levels.