uninvestigated, family history and the observed phenotype makes the differential diagnoses of congenital abnormality related to choroidal inflammation, retinotoxic medication uses, infective, inflammatory, systemic metabolic or choroidal vasculopathy highly improbable.Other causes of well-defined atrophy such as this occurs in choroideremia, gyrate atrophy, and bifocal choroidal atrophy. However, none of these are consistent with this case. In summary, we present a novel lobular chorioretinal dystrophy for discussion.