A 13-year-old girl was found to have a "morning glory" optic disc anomaly associated with remnants of the primitive hyaloid vasculature, midline cleft lip and palate, agenesis of the corpus callosum, and a sphenoidal encephalocele. The association of these developmental anomalies indicates that the "morning glory" optic disc anomaly may occur as part of a more extensive syndrome of midline cranioencephalic dysraphism.