Three cases of cranial vault defects in fetuses are described. All 3 cases underwent postmortem examination, and in 2 of them postmortem magnetic resonance imaging was also performed. The phenotypical differences between the cases illustrate a spectrum of cranial vault defects, associated anomalies, and pathogenic mechanisms involved in the etiology of this rare malformation. All 3 cases were male, and the cranial vault defects corresponded to exencephaly with acrania/acalvaria caused by amniotic band syndrome, acalvaria, and acrania presenting in a chorangiopagus parasiticus twin.