Reported is a 4-year-old female with Chiari II malformation and congenital hydrocephalus associated with lumbosacral myelomeningocele who also presented with frontal calvarial foramina depicted by head computed tomographic scans with three-dimensional reconstructions. Magnetic resonance demonstrated bilateral and symmetric frontal lobe malformation-separated-lip clefts schizencephaly-adjacent to the bilateral frontal calvarial foramina. Because the different types of malformations evident in this patient do not share the same developmental origin, the association of findings is unusual and suggests the occurrence of an unknown prenatal factor acting along different stages of central nervous system development.