We report two patients with transient visual symptoms after an acute illness (hypoglycemia and head trauma) who went on to develop a progressive neurodegenerative disease (adrenoleukodystrophy). This report supports the suggestion that environmental factors play a role in the initial expression of the disease and reviews atypical visual manifestations of adrenoleukodystrophy. Proper diagnosis and institution of early treatment before the catastrophic deterioration typical of its natural history requires a high degree of suspicion.