Anencephaly is a severe anomaly of the brain that results from the failure of the cephalic part of the neural tube to close during the fourth week of pregnancy. In anencephaly, the mortality rate is 100% during intrauterine life or within hours or days after birth. Its prevention is based on supplementation with folic acid during pregnancy. We report a case of complicated anencephaly with death of the fetus in the womb of a young woman who didn’t take folic acid during her pregnancy.