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A.Ü.Tıp Fak.Göz Hastalıkları Anabilim Dalı Macular halo is a clinical finding of ring-like crystalloid opacities around the fovea with no visual impairment. Since it is associated with sphingomyelinase deficiency, the entity is classified as a variant of Niemann-Pick disease. This paper presents a 16 year-old female patient with macular halos and systemic association of Niemann-Pick type B disease which is a rare entity with unique macular involvement. Keywords : Niemann-Pick disease, lipidosis, macular halo