Abstract: Background: Anencephaly is a neural tube defect (NTD) which is due to defective closure of cranial neuropore. This NTD is associated with other congenital anomalies in most of the cases. This study was undertaken to determine the incidence of anencephaly among congenital malformations seen at autopsy. The study aims to identify the risk factors associated with anencephaly in our population. Attempt was made to correlate the incidence with associated systemic anomalies, maternal age, birth order and sex of the fetus.