To report clinical and imaging findings of a patient with unilateral choroidal excavation in the macula detected by spectral-domain optical coherence tomography (SD-OCT) in Best Vitelliform Macular Dystrophy.

The clinical and imaging characteristics of a 35 Year old woman with best vitelliform macular Dystrophy was assessed using SD-OCT, Fluorescein angiography (FA) , Fundus autoflourecence and Indocyanine green angiography (ICG).

The visual acuity was 20/25 in the right eye and 20/20 in the left eye. SD-OCT demonstrated a focal macular choroidal excavation in the right eye and revealed disturbance of the RPE and photoreceptors layers and small subretinal fluid without obvious excavation in the left eye. Fundus autoflourecence images of the both macular region showed an outer circumferential ring of hyperfluorescent. Fluorescein angiography (FA) of the both eyes revealed irregular hyperfluorescence in early phase corresponding to the RPE attenuation over the macular area without leakage. Indocyanine green angiography (ICG) of both eyes was near normal.

To the best of our knowledge, our patient is the second report of choroidal excavation in Best disease.Finally there may also be an association or a Causal relationship between best disease and FCE, which requires further studies to confirm.