Acrania-exencephaly-anencephaly sequence

Acrania–exencephaly–anencephaly sequence is rare forms of neural tube defects. The progression is from a relatively normal-appearing exposed brain due to an absent cranium called as acrania, to an amorphous brain mass term as exencephaly. We present a case of 20 years female, nonconsaguinous marriage, G1P0A0 having amenorrhea since 3 months. On ultrasonography, a single fetus of gestational age of about 15 weeks gestation showed congenital malformation- acrania with exencephaly. Final impression on fetal autopsy given was acrania–exencephaly with deformed brain, ill-formed cervical C1, 2 spine, bilateral pulmonary immaturity- Grade II with pulmonary congestion and hemorrhage. We are presenting this rare fetal anomaly of acrania–exencephaly–anenecephaly sequence for its clinical, radio imaging, and fetal autopsy findings.