Pigmented Paravenous Retinochoroidal Atrophy (PPRCA) is a rare disorder of unknown etiology, characterized by perivenous pigment accumulation associated with peripapillary and radial zones of retinal pigment epithelial atrophy that are distributed along the retinal veins, commonly bilateral and symmetric. The diagnosis of PPRCA is based on typical and characteristic fundus appearance.

Vision is rarely affected and it is an incidental finding. Color vision is unaffected. Macular involvement is rare but cystoid macular edema have been reported.

Although the etiology of PPRCA is unknown, inflammatory and infectious causes have been reported and it is possible that it has a genetic basis.

The differential diagnoses include chorioretinal degeneration and inflammatory diseases that cause chorioretinal atrophy.