The incidence of lacrimal gland adenocarcinoma is low. This study was designed to analyze the clinical and prognostic characteristics of lacrimal gland adenocarcinoma.This was a clinical study and literature review; 25 patients diagnosed with lacrimal gland adenocarcinoma by histopathology were enrolled and their medical history data were collected.The incidence of bone destruction and surrounding tissue invasion was 52% and 44%, respectively. The incidence of distant metastasis of lacrimal gland adenocarcinoma was about 50%. The 5-year overall survival rate of death or metastasis was 33.5%. Age, sex, laterality, tumor size, pathology type, bone destruction, nerve or perineural invasion, invasion of peripheral tissue, T stage, AR, Her-2 and treatment had no significant correlation with lacrimal adenocarcinoma's prognosis (P > 0.05), while the higher expression of Ki-67 may have higher risk of death or metastasis (P = 0.020).The incidence of bone destruction and distant metastasis of lacrimal adenocarcinoma is high and the imaging examination is necessary to assess the risk of distant metastasis. The 5-year survival rate of death or metastasis is 33.5% and the high expression of Ki-67 predicts poor prognosis of lacrimal adenocarcinoma.© 2024. The Author(s).