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Background: The lacrimal caruncle, a nodular structure located at the inner canthus, contains a variety of tissue types, leading to diverse clinical manifestations of its lesions. Caruncular lesions, though rare, may be benign or malignant, complicating diagnosis and treatment. This systematic review aims to provide a comprehensive overview of the clinical profiles, histopathological features, and management strategies for caruncular lesions., Methods: This was a PROSPERO-registered (CRD42024529956), PRISMA guideline-adherent systematic review. Databases searched include PubMed, Scopus, and CINAHL Ultimate from inception to June 29, 2024. Grey literature was also searched. Studies involving patients diagnosed with caruncular lesions were included. A novel classification strategy has been developed and proposed., Results: From an initial 1602 articles, 16 studies were included, representing a range of demographics and clinical features. Histopathological diagnosis varied widely, with benign lesions more common than malignant ones. Common benign lesions included nevi, papillomas, and pyogenic granulomas, while malignant lesions included basal cell carcinomas, sebaceous cell carcinomas, and melanomas. Medical management often combined topical therapies with surgical excision. Surgical management primarily involved excision, with some cases necessitating more radical procedures, which were typically reserved for extensive or malignant lesions. Recurrence was noted in several studies, with melanomas being at the highest risk of recurrence., Conclusion: Caruncular lesions exhibit diverse histopathological profiles, necessitating a combination of clinical and histopathological examination for accurate diagnosis and management. Early detection and appropriate management are crucial for improving patient outcomes. Despite the rarity of these lesions, their potential malignancy and systemic metastasis create a need for prospective studies., Competing Interests: Competing interests: The authors declare no competing interests. Ethical approval: We confirm that we have read the Journal’s position on issues involved in ethical publication and affirm that this report is consistent with those guidelines., (© 2024. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)