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Walaa Alturkistany,1,2 Rawan Althaqib,1 Naif Alsulaiman,1 Azza Maktabi,3 Sahar Elkhamary,4 Hailah Alhussain1 1Oculoplastics Department, King Khalid Eye Hospital, Riyadh, Saudi Arabia; 2Ophthalmology Department, King Abdulaziz University Hospital, Jeddah, Saudi Arabia; 3Pathology and Laboratory Medicine Department, King Khalid Eye Hospital, Riyadh, Saudi Arabia; 4Diagnostic Imaging Department, King Khalid Eye Hospital, Riyadh, Saudi ArabiaCorrespondence: Walaa Alturkistany Tel +966504199412Fax +966114821908Email walturkistany@gmail.comPurpose: To study the demographics and clinical presentation of biopsied lacrimal gland lesions in a tertiary eye center and determine the accuracy of radiological imaging and blood investigations in reaching the diagnosis. We also studied the histopathological outcome of different lacrimal gland biopsy approaches.Materials and Methods: A retrospective review of patients’ charts from 2014 to 2021 who underwent lacrimal gland biopsy. The data collected included demographics, clinical presentations, surgical approaches used, blood workup done, and histopathological and radiological diagnoses. The accuracy of radiological imaging in diagnosing lacrimal gland pathologies was evaluated by comparing radiological interpretation to the final pathological report.Results: In a total of 48 cases, the most common lacrimal gland lesion was nonspecific inflammation of the lacrimal gland (34%, n=17) followed by lymphoproliferative lesions (25%, n=12). Females (62.5%, n=30) were more affected than males (37.5%, n=18). Other lesions included pleomorphic adenoma, angiolymphoid hyperplasia with eosinophilia, and IgG4 disease. Complete blood investigations were performed in 45.8% of patients and a systemic disease was found in only 18.2%. The initial assumptive diagnosis based on MRI and CT scan images was congruous with the histopathological diagnosis in 42% and 12%, respectively. Three surgical approaches were used for incisional biopsy in this study