UNLABELLED: Elevated Immunoglobulin G4 (IgG4) levels have been implicated in various autoimmune and inflammatory diseases, including those affecting the nasopharynx and laryngopharynx. The current systematic review and meta-analysis aimed to determine the prevalence of elevated IgG4 and associated risk factors in patients with nasopharynx and laryngopharynx diseases. This systematic review and meta-analysis were conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A comprehensive search of PubMed, Embase, Web of Science, and Scopus databases was performed from inception until July 2024. Keywords and Medical Subject Headings (MeSH) terms A random-effects model was used to pool prevalence estimates, and heterogeneity was assessed using the SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12070-024-05189-3.