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Abstract The corneal endothelium plays an integral role in regulating corneal hydration and clarity. Endotheliitis, defined as inflammation of the corneal endothelium, may disrupt endothelial function and cause subsequent visual changes. Corneal endotheliitis is characterized by corneal edema, the presence of keratic precipitates, anterior chamber inflammation, and occasionally limbal injection, neovascularization, and co-existing or superimposed uveitis. The disorder is classified into four subgroups: linear, sectoral, disciform, and diffuse. Its etiology is extensive and, although commonly viral, may be medication-related, procedural, fungal, zoological, environmental, or systemic. Not all cases of endothelial dysfunction leading to corneal edema are inflammatory in nature. Therefore, it is imperative that practitioners consider a broad differential for patients presenting with possible endotheliitis, as well as familiarize themselves with appropriate diagnostic and therapeutic modalities.