Purpose: To evaluate serum and aqueous humor levels of fetuin-A in patients with pseudoexfoliation syndrome (PEXS) in comparison with those of age- and sex-matched healthy subjects.Materials and Methods: This prospective study included 25 patients with PEXS and 25 control subjects who were undergoing cataract surgery without any systemic or ocular disease. Aqueous humor and serum fetuin-A levels were measured with enzyme-linked immunosorbent assay method.Results: The mean age of the PEXS group (14 males, 11 females, n = 25) was 57.7 6.9 years, and the control group (13 males, 12 females, n = 25) was 58.1 5.7 years. There was no difference between the groups in terms of age (p = 0.77) and sex (p = 0.83).The mean serum fetuin-A level of the PEXS group did not differ from that of the control group (p = 0.53). The mean aqueous humor level of the PEXS group was significantly higher than that of the control group (p = 0.032). There were no significant correlations between aqueous humor and serum fetuin-A levels among patients with PEXS and control group (p > 0.05).Conclusions: Increased levels of fetuin-A in aqueous humor of patients with PEXS may show the local effect of fetuin-A on the anterior segment. With considering the wide range of possible biological functions of fetuin-A in the pathogenesis of PEXS, further studies are needed to clarify the clinical relevance of these findings.