To report the youngest case of Pigmented paravenous retinochoroidal atrophy (PPRCA).

27 month old baby presented to clinic because of right eye deviation and in funduscopy the pigmented atropht around veins were evident.Electroretinogram done to rule out other retinal pathologies.

the diagnosis confirmed to be Pigmented paravenous retinochoroidal atrophy (PPRCA).It is an uncommon disease characterized by “perivenous aggregations of pigment clumps” associated with peripapillary and radial zones of retinochoroidal atrophy that are distributed along the retinal veins.Patients are usually asymptomatic and the disease process is non-progressive or slow and subtly progressive.It is commonly bilateral and symmetric.Pigmented paravenous retinochoroidal atrophy (PPRCA).Young men are most commonly affected with only 10 cases reported in females.It was first proposed by Franceschetti and reported patients have been between 4-25 years old.To our knowledge this was the youngest PPRCA ever reported.

This is the youngest case of Pigmented paravenous retinochoroidal atrophy (PPRCA) reported. It is very important to know uncommon retinal disease to prevent further additional work ups and to make sure the parents about the benign natural cours of the disease.