This 14 year old male has a chief complaint of a sudden reduction of visual acuity in the right eye. His visual acuity has been normal in both eyes in the past.

The clinical features favoring the diagnosis of autosomal dominant Best disease in this patient include the bright yellow vitelliform lesions, the relatively good visual acuity in the left eye and the extensive autosomal dominant family history. Autosomal dominant macular dystrophies often have good acuity despite a fairly dramatic fundus appearance.