Monoamniotic twins, although uncommon, are associated with high antenatal and perinatal morbidity and mortality. The associated complications include cord entanglement, congenital anomalies, twin-twin transfusion syndrome, intrauterine growth retardation and preterm delivery. A thorough literature search using Medline and OVID was performed to look at the current diagnostic criteria for monoamniotic twins and the subsequent management. Specific criteria are employed during ultrasonography for making the diagnosis. Different opinions in the literature exist regarding the proper antepartum monitoring of monoamniotic twins, the optimal timing and the mode of delivery. Generally, it is agreed that there should be regular antepartum fetal heart trace monitoring and serial sonograms with umbilical artery Doppler flow study. Most studies advocate delivery at 32 weeks to reduce the risk of intrauterine deaths related to cord entanglement. Cesarean section is the preferred mode of delivery even though cases of successful vaginal delivery have been reported.