Best Disease also known as Vitelliform Macular Dystrophy (VMD), classically presents with defective central vision. Macula which is responsible for the central vision and colour perception is affected. Individuals affected by Best disease, initially have normal vision followed by decrease in central visual acuity as well as metamorphopsia. The case presented is of 45-years-old female with complaints of gradual progressive defective Vision of over 2 years . A well-circumscribed single greyish lesion of size 1.5 DD was noted in the macula, which was confirmed to be Best disease.