There is a spectrum of disorders with ocular and neurological manifestations that overlap those of our patient. In the congenital muscular dystrophies, an underlying defect in glycosylation is thought to result in severe defects in neuronal migration, thus causing hypoplasia of various brain and eye structures.2 Walker-Warburg syndrome is the most severe, with brain abnormalities including lissencephaly, hydrocephalus, cerebellar malformation, hypomyelination of the white matter, and agenesis of the corpus callosum.3 Ocular posterior segment abnormalities include retinal dysplasia as well as hypoplasia or atrophy of the optic nerve and macula. Lissencephaly can also be found in Fukuyama congenital muscular dystrophy and muscle-eye-brain disease with associated ONH and retinal hypoplasia.4 A case of genetically proven muscle-eye-brain disease with ONH and peripheral retinal nonperfusion with secondary fibrovascular proliferation and retinal detachment was recently described.2 Our patient shares many similar features but had no evidence of a muscular dystrophy, with clinically absent hypotonia and a normal creatine kinase level. Additionally, a case of de Morsier syndrome with similar ocular findings of bilateral peripheral retinal nonperfusion and neovascularization with resultant retinal detachment was reported recently.5 We suggest that the abnormal neuronal development that led to lissencephaly resulted in optic nerve and retinal maldevelopment and subsequent associated retinal vascular maldevelopment. Tractional retinal detachment is an end stage of a process that starts with nonperfusion and progresses to ischemia and extraretinal fibrovascular proliferation in a variety of pediatric retinal diseases.