Aim: To evaluate whether aqueous humor levels of pigment epithelium-derived factor ( PEDF) are increased in patients with uveitis Methods: Aqueous humor levels of PEDF and tumour necrosis factor alpha ( TNF alpha) were determined by ELISA in 34 uveitis samples and 9 cataract control samples. Results: Aqueous humor PEDF and TNF alpha levels were significantly higher in patients with uveitis than in controls ( mean ( SD) 6.4 ( 0.8) v 1.3 ( 0.2) mu g/ml and 14.7 ( 3.8) v 4.2 ( 0.4) pg/ml, respectively; p < 0.01). A positive correlation between PEDF and TNF alpha was found in patients with uveitis ( r = 0.40, p < 0.01). Furthermore, PEDF levels in aqueous humor were increased in proportion to the disease activity of uveitis. Conclusion: The results show that aqueous humor levels of PEDF are increased in patients with uveitis. Our observations suggest that aqueous humor levels of PEDF may be increased as a countersystem against inflammation in uveitis.