A case of amniotic band syndrome (ABS) presenting with acalvaria is reported. ABS includes a spectrum of non-genetic anomalies, varying from simple digital band constriction to major craniofacial and visceral defects, and even fetal death. Acalvaria is a rare congenital malformation characterised by the absence of the dome-like superior portion of the cranium comprising the frontal, parietal, and occipital bones and dura mater, in the presence of a normal skull base and facial bones with complete cranial contents. No two cases are the same. Acrania or absence of the flat skull bones with disorganised cerebral hemispheres have been reported in the presence of amniotic bands. ABS is an aetiological factor in acalvaria. Appropriate counselling for affected families needs to be given after prenatal diagnosis.
