Introduction: Anencephaly also called cranioschisis is part of neural tube defects spectrum which occur when the neural tube fails to close normally during the 3rd and 4th weeks of development leading to fetal loss, still birth or neonatal death. Literature show that causation of anencephaly is multifactorial involving interaction of genetics and the environment though not well characterized. In this case report, we present the features of anencephaly and its different clinical associated malformations. Methods: A case report study design was employed to explore the prenatal and antenatal events that resulted into an anencephaly delivery. The intra-natal and immediate postnatal fetal observations and outcome were documented. We also documented the care given to the mother throughout ANC, time of delivery and postnatally until discharge. Results: 29-year G2P1+0 at 29W2D, presented with 2 days’ sudden progressive per vaginal bleeding and clear non-foul discharge on 2nd day of admission with no history of abdominal pain, fever or trauma. Two days later she was delivered by Caesarean section to a grossly neural malformed preterm baby boy with APGAR score 6 at 1 minute and 4 at 5 minutes. Birth weight and length were 1.5 kg and 48.6cm respectively. The baby life indicators deteriorated progressively and finally died at 36 minutes after time of delivery. Conclusion: Although interaction between genomic and environmental factors that play a key role in the causation of anencephaly can not clearly be evaluated, there is an understanding of pre-natal and antenatal factors that predispose to this case such as lack of Folate and or interference with its bioavailability, use of teratogenic drugs taken during pregnancy and antenatal maternal conditions. We therefore recommend routine supply of folate to girls and women intending or risk to conceive 3 months before pregnancy through first trimester and health education about use of native drugs and any other conventional medicines during pregnancy.