Background: Amniotic Band Syndrome is a congenital disorder presenting with fetal anomalies associated with fetal placental fibrous bands that may cause disruptions, deformations or malformations without a consistent anatomical pattern. Anomalies are limited to external structures with or without body wall disruption or internal malformations that vary in severity and location. These malformations are characterized by being asymmetric, polymorphic and not respecting any embryological systematization. The pathophysiology of this syndrome is the subject of a debate that started more than seventy years ago and continues to this day. Case presentation: We report here two cases of amniotic band syndrome. The first one is that of a 22-year-patient admitted for delivery at 37 weeks of amenorrhea. Newborn examination at birth revealed the presence of upper limb ischemia, whose therapeutic decision following the unfavorable evolution towards necrosis with gangrene was the amputation of the upper limb. The second case is that of a 29-year-old woman pregnant at 27 weeks, whose ultrasound scan showed the presence of anencephaly associated with a myelomeningocele adherent to the placenta. The therapeutic decision was to convene the ethics committee to agree to the medical termination of pregnancy, which was deemed admissible given the lethality of the malformation. Discussion: Amniotic Band Syndrome includes a wide variety of fetal anomalies associated with fibrous bands that entangle or constrict various parts of the fetus or umbilical cord in utero. Despite numerous studies, no pathogenesis theory has been proven to date. Diagnosis is possible as early as the first trimester, depending on the nature and severity of the malformations observed. The therapeutic decision varies according to the case. Conclusions: ABS is uncommon but screening should be systematic in the neonatal period. In order to improve diagnosis, it is necessary to promote antenatal screening reference centers that assess suspected cases of rare malformations.