Conjunctival lymphangiectasia is a low-incidence ocular surface disease that is currently rarely reported in the relevant literature. It may be related to cosmetic eyelid surgery, tumor, radiation or chemotherapy and other factors and often causes a foreign body sensation, lacrimation, eye pain, visual fatigue and other discomfort. These symptoms of constant eye irritation affect the patient's quality of life. At present, anterior segment optical coherence tomography can be used for clinical diagnosis, and the novel monoclonal antibody D2-40, as a marker of lymphatic endothelial cell dilatation, has high specificity in pathological diagnosis. Previous studies have not fully defined the pathogenesis of the disease, and treatment methods vary. Conventional treatment has resulted in varying degrees of damage to the conjunctiva in patients. In recent years, anti-vascular endothelial growth factor drugs have been reported to be effective in treating the disease with few complications. This article reviews the pathogenesis, diagnosis and treatment of this rare disease in order to gain a better understanding of conjunctival lymphangiectasia and provide more support for clinical diagnosis and treatment.