AIM: To analyze the clinical and pathological features of 89 patients presenting as lacrimal gland prolapse (LGP). METHODS: This retrospective study included 89 patients presenting as LGP. Magnetic resonance imaging (MRI) scan was performed for all patients. Pathology and immunohistochemical staining of prolapsed tissue were performed during the surgery. The histopathological subtype was obtained, and the related clinical manifestations of different subtype were marked. RESULTS: Among the 89 patients involved, the histopathological subtype includes dacryoadenitis (43%; n=38), focal lymphocytes infiltration (20%; n=18), immunoglobulin G4 (IgG4)-related lacrimal gland inflammatory disease (15%; n=13), lacrimal gland (13%; n=12), and extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (9%; n=8). As for manifestations of different subtypes, eyelid swelling was found the most frequent of lymphocytes infiltration (44%, n=8), and palpable lacrimal gland mass of dacryoadenitis (55%, n=21). All the IgG4-related lacrimal gland inflammatory disease (100%, n=13) and most dacryoadenitis (97%, n=37) presented as bilateral. CONCLUSION: LGP has the histopathological subtype most commonly as inflammation, followed by structural and lymphoproliferative changes. Most of patients present as eyelid swelling. Clinical manifestations can be significant to differentiate the diagnosis.