VSX1 (Visual System Homeobox 1) is a homeodomain transcription factor critical in ocular development, particularly in the cornea and retina. It regulates genes involved in cell differentiation and tissue homeostasis. Mutations in the *VSX1* gene have been linked to posterior polymorphous corneal dystrophy (PPCD) and keratoconus, though its exact pathogenic mechanisms remain debated. VSX1 antibodies are essential tools for studying its expression, localization, and functional roles in both developmental and disease contexts. These antibodies, often generated in rabbit or mouse hosts using immunogenic peptide fragments, enable detection of VSX1 protein via techniques like Western blotting, immunohistochemistry, and immunofluorescence. Research using VSX1 antibodies has revealed its expression in corneal endothelial cells, retinal bipolar cells, and embryonic ocular tissues, supporting its role in maintaining corneal transparency and retinal circuitry. However, inconsistencies in antibody specificity and cross-reactivity have occasionally led to conflicting data, underscoring the need for rigorous validation. Recent studies also explore VSX1's potential involvement in epithelial-mesenchymal transition (EMT) pathways in corneal disorders. As genetic therapies for inherited corneal diseases advance, VSX1 antibodies remain vital for validating gene-editing outcomes and understanding genotype-phenotype correlations.