Purpose: To present the ocular pathology of a three-year-old child wit
h Leber's congenital amaurosis (LCA) who died from an unrelated episod
e of presumed viral meningitis. Methods: Autopsy was performed, and in
addition the globes were harvested for pathological examination. Befo
re the child's death, known systemic disorders with infantile retinal
dystrophy were excluded. Results: The outer nuclear layer and photorec
eptor layers are the primary site of retinal pathology in LCA. Conclus
ion: This report of the pathological findings in LCA, early in the nat
ural history of the disease, indicates that process appears to be a de
generation rather than an agenesis.