Objective: The aim of this study is to find out the rate of occurrence, management and diagnosis of the anencephaly. Methodology: This was a prospective transverse research work conducted in a period of one year from June 2018 to May 2019 in Gynecology Department of DHQ Teaching Hospital Dera Ghazi Khan. Total 13 females with pregnancy having fetuses with the complications of Neural Tube Defect who got admission in the hospital were the part of this research work. We evaluated the establish diagnosis, various risk factors and rate of occurrence of anencephaly in this research work. Results: The rate of incidence of the anencephaly was about 3.20 per one thousand deliveries and spina-bifida was two per thousand deliveries, we carried out the diagnosis of 100.0% anencephalic and 80.0% infants with the spina-bifida with the utilization of ultrasonography. The termination of the pregnancy carried out in sixty-two percent patients though normal delivery route of vagina. Total 12.0% female patients were present with diabetes. There was dominancy of the female gender in the anencephalic fetuses as 61.5%. Conclusion: Developed maternity cares, with the facilities for prenatal methods of diagnosis for the identification of the anencephaly and timely termination of abnormal fetuses can support to the prevention of this inherited problem with its related high rate of mortality. The supplementation of the folic acid prior from pregnancy and in the first three months of pregnancy can decrease the occurrence of this very complication. KEYWORDS: Maternity, Vagina, Delivery, Termination, Prenatal, Anencephaly, Diabetes, Abnormal, Complication, Folic Acid, Occurrence, Fetus, Supplementation.