OBJECTIVE: To document the presenting ocular signs and the clinical course of Golden Retriever dogs with a progressive anterior uveitis, often associated with the histologic presence of iridociliary cysts. Animals studied Seventy-five Golden Retriever dogs (142 affected eyes) referred to a private practice referral ophthalmology clinic between 1994 and 1999. Procedures Complete ophthalmic evaluation with slit-lamp biomicroscopy, indirect ophthalmoscopy, applanation tonometry, and gonioscopy. Hematology, serum biochemical evaluations, and serologic titers for endemic infectious agents were also used in selected cases. RESULTS: The age range of affected dogs was 4.5-14.5 years, with a mean age of 8.6 +/- 2.1 years. The majority of the dogs (n = 66) were affected in both eyes at first presentation. The sex distribution included 4 intact males, 32 neutered males, and 39 spayed females. Hematology, serum biochemical evaluations, and serologic titers for endemic infectious agents failed to demonstrate any underlying disorder. The ophthalmic hallmark of this syndrome was the appearance of pigment on the anterior lens capsule, often in a radial orientation. This capsular pigment was seen both with and without associated uveal cysts. Although single to multiple iridociliary cysts were noted clinically in only 13.3% of the cases, cysts were common on histopathology of advanced glaucomatous, blind eyes. Fibrin was observed in the anterior chamber of 37% of the cases, and often was a precursor for glaucoma. Cataract formation (37%) and glaucoma (46%) were frequent sequelae to the uveitis. Posterior synechiae formation occurred in 50% of the cases. Histopathologic analysis of four enucleated eyes and the eviscerated specimens from 14 glaucomatous eyes demonstrated thin-walled iridociliary epithelial cysts in 3/4 and 12/14 cases, respectively. Microscopically, little to no uveal inflammatory infiltration was commonly noted. Conclusion The overall prognosis for this progressive uveitis in Golden Retriever dogs is guarded, with 46% of the eyes becoming blind due to glaucoma. Iridociliary cysts in Golden Retriever dogs may lead to the development of glaucoma in this breed.