AIM: To evaluate the clinical features of primary lacrimal gland pleomorphic adenoma (LGPA). METHODS: This was a 2-center, retrospective, observational study of primary LGPA patients who underwent tumor resection. Ophthalmic examinations and orbital computed tomography (CT) and/or magnetic resonance imaging (MRI) were performed. RESULTS: Totally 18 patients (10 males and 8 females, mean age 56.3±13.8y) were enrolled. Initial symptoms were unilateral proptosis in 8 patients, diplopia in 3 patients, and pressure sensation and no chief complaint in 2 patients each. The best-corrected visual acuity of the affected eye was 0.26±0.44 logMAR, and the intraocular pressure (IOP) of the affected and healthy eyes was 20.1±9.9 and 15.8±4.3 mm Hg, respectively. The difference in degree of proptosis between the affected and healthy eyes was 4.1±2.2 mm based on the Hertel ocular protrusion meter. One case had a history of breast cancer. Seventeen of 18 patients with an orbital lacrimal gland origin underwent total tumor excision by anterior/lateral orbitotomy. CONCLUSION: Orbital LGPA can complicate IOP elevation, and require total tumor excision by orbitotomy. In rare cases, systemic malignancy may complicate LGPA, and in such cases total removal of the lacrimal gland tumor should be considered at the initial surgery.