Leber's congenital amaurosis (LCA) is a group of rare inherited retinal dystrophies characterized by vision loss, often from birth. LCA is the most severe form of all inherited retinal dystrophies, accounting for at least 5% of these, and is one of the main causes of blindness in children. Diagnosis is based on clinical history of failure to develop reactions to visual stimuli, roving eye movements or nystagmus, sluggish pupillary responses and a normal, or less frequently, an abnormal fundus on dilated fundoscopy.