Patient: 32 years of age, female, BCVA 0.5 at OD, 1.0 at OS; IOP 17/14 mmHg. General Medical History: empty. Ocular Medical History: deterioration of visual acuity at OD since 3 weeks. Main Complaint: metamorphopsia at OD. Purpose: to present retinal changes in Best's vitelliform Dystrophy. Methods: Colour Fundus Images, Image Fundus Autofluorescence (HE), SD-OCT (HE), Fluorescein Angiography FFA (HE), Electro-oculogram EOG. Findings: Colour fundus images: presenting deposits of lipofuscin on Bruch's membrane and a scarring of the macula. OCT: showing deposits of lipofuscin on Bruch's membrane (*), correlating with lipofuscin and melanofuscin granulae in the RPE. Photoreceptor layer has lost the outer segments (#). Image fundus autofluorescence: localised areas of hyperfluorescence centrally in the fovea depicting lipofuscin accumulation in the RPE. Electro-oculogram EOG: showing reduced Arden ratio of 1.16 at OD and 1.1 at OS. Fluorescein Angiography FFA (HE): showing rarefication of foveolar capillaries, no leakage of RPE. Discussion: Best's disease is a hereditary affection with juvenile onset. The fundus may present various stages. In a paper of Lorenz and Preising (1) relevant data are summarized . They showed, that advanced stages discloses deposits of lipofuscin on Bruch's membrane, correlating with lipofuscin and melanofuscin granulae in the RPE. Photoreceptors have lost the outer segments. Usually the loss of function correlates with a reduced Arden ratio of the electro-oculogram. They stated, that Best's disease is caused by mutations in VMD2 (hBEST1), producing Bestrophin, which is a regulatory part of a Ca(2+) channel or Cl(-)channel. Literature: (1) Lorenz B, Preising MN. Best's disease. Overview of pathology and its causes. Ophthalmologe. 2005 Feb;102(2):111-5.