Despite the rarity of lacrimal gland adenoid cystic carcinoma (LGACC), it is the most common malignant epithelial cancer of the lacrimal gland, with a low survival rate.In selected cases, the combined approach of globe-sparing surgery with adjuvant radiotherapy is considered to have a favorable visual and functional outcome with good local control rates.Future randomized clinical trials are crucial to determine the outcomes between conservative and radical surgical protocols.The therapeutic strategy of LGACC relies on a strong multidisciplinary collaboration between an orbital surgeon, radiotherapist, pathologist, and oncologist.