Background: Anencephaly is a rare but lethal congenital anomaly of the neural tube. Ideally, the diagnosis is made early in pregnancy and the pregnancy is usually terminated. This is to prevent avoidable complications during pregnancy and childbirth as well as the accompanying psychological trauma from late intrauterine foetal death or death during the neonatal period. Objective: To report a case of anencephaly in an unbooked primipara at 35 weeks gestation. Methods: The case note of the patient and how she was managed were reviewed. A relevant review of the literature on the subject was also done. Case report: Mrs A.N was a 22year old unbooked G3P1+1 who was referred to the Rivers State University Teaching Hospital (RSUTH) on the 4th of September, 2021 with a three-day history of bleeding per vaginam and an ultrasound scan report of an absent cranium and club foot at 35weeks gestation. She resided in a rural area and was on herbal medication in the index pregnancy. She did not receive routine antenatal medications and had no family history of congenital malformations. She had an induction of labour at presentation and delivered a severely asphyxiated male anencephalic baby weighing 2.0kg. The baby died 9minutes after delivery. Conclusion: Anencephaly is a lethal anomaly that is associated with folic acid deficiency. Despite available diagnostic tools, most women in rural areas lack access to antenatal care and will have a late diagnosis and increased morbidity. Thus, there is a need to improve access to antenatal care for women in rural communities, as well as supplement food with folic acid for women in the reproductive age group.