Background: Anencephaly occurs due to the complete absence of cranial vault and subsequent disruption of the cerebral cortex with a severely damaged brain. In anencephaly, the forebrain and brain stem are exposed. Forebrain either does not develop or is destroyed, leading to the absence of cerebrum and cerebellum. Methodology: Neural tube defects were taken in the study group. During the autopsy, clinical findings, external examination, internal examination, and photography were done along with the histopathology of the specimens to confirm the anomalies at microscopic level using hematoxylin and eosin staining. Results: In our study, we observed a simian crease in 4 out of 5 (80%) cases. Furthermore, there was presence of tooth which was not seen in previous studies. Central nervous system anomalies like spina bifida, gastro intestinal tract (GIT) anomalies like cleft palate, intestinal obstruction of megacolon, and malrotation of gut were some of the common anomalies which were observed in our study. Conclusion: It may be suggested that Anencephaly shows a female predisposition and the cases seems to be associated more in the primigravida females.The classical phenotypic presentation of anencephaly having absent cranial vault ,low set ears, protruding eyes were present in all subjects studied. In our study, we observed a simian crease in 4 out of 5 (80%) cases. Furthermore, there was presence of tooth which was not seen in previous studies. Central nervous system anomalies like spina bifida, GIT anomalies like cleft palate, intestinal obstruction of megacolon, and malrotation of gut were some of the common anomalies which were observed in our study.