Monoamniotic twin pregnancies are rare, but early diagnosis of such pregnancies is critical, as the incidence of complications in these pregnancies is much higher than in diamniotic or dichorionic twin pregnancies. Overall, only 70% of all monoamniotic twins will survive. Furthermore, approximately half of fetal deaths in these pregnancies are because of the high incidence of fetal anomalies (15%-25%), such as twin reversed arterial perfusion sequence and conjoined twinning. Therefore, early anatomy screening in the first trimester of pregnancy is recommended. Other causes of fetal death in these pregnancies include twin-twin transfusion syndrome, tight cord entanglement, or acute hemodynamic imbalances through the large placental vascular anastomoses. After viability, fetal surveillance can be intensified, as this decreases the risk of in utero death. Both inpatient and outpatient surveillance are reasonable. If otherwise uncomplicated, monoamniotic twins should be delivered at 33 to 34 weeks' gestation. Most centers will deliver by cesarean delivery, but some continue to advocate for vaginal delivery. Lastly, neonatal morbidity is high in monoamniotic twin pregnancies and is mainly related to prematurity.