Background <p>Idiopathic Intracranial Hypertension (IIH) is a disease of unknown etiology. Understanding the role of Retinol Binding Protein (RBP) in IIH pathogenesis could provide valuable perceptions into disease mechanisms and potentially identify new therapeutic targets.</p> Methods <p>The study conducted a comprehensive search of electronic databases following PRISMA guidelines. The inclusion criteria involved human studies published in English that reported RBP levels in IIH patients. The New Castle Ottawa scale was used for the quality assessment of the included studies. Analysis was done using the Random Effects Model with 95% Confidence Interval.</p> Results <p>A total of 4 studies met the inclusion criteria for meta-analysis. The quality score for the included studies ranged from 5 to 6. The pooled analysis indicated significant association of RBP with risk of IIH, with higher serum RBP levels (SMD = 1.56, 95% CI: 0.13 to 2.98, I² = 93.9) and lower CSF RBP levels (SMD = -0.67, 95% CI: -1.37 to 0.04, I² = 65.4) as compared to controls. Significant heterogeneity was found in the studies, suggesting variability in study design and methods. Further subgroup analysis by investigation method also showed significant results (SMD = 1.56, 95% CI: 0.13 to 2.98, I2 = 93.9).</p> Conclusion <p>The findings of this study propose that an elevated level of RBP may contribute to the risk of IIH, potentially through its involvement in retinol transport and metabolism. The study has been registered in PROSPERO (CRD42023436827).</p>