In monochorionic monoamniotic twin pregnancies, two fetuses share a single placenta and a single amniotic cavity. This unique anatomic configuration, although rare, puts them at high risk for a number of complications including structural anomalies, twin-twin transfusion syndrome, selective intra-uterine growth restriction and cord accidents. Early diagnosis and patient counselling are therefore crucial. Level 1 evidence on the optimal management of monoamniotic twins is lacking, but observational studies provide some guidance for best practice. In uncomplicated monoamniotic twins, after viability, fetal mortality is less than 5% if close surveillance is initiated. This can be done in either an inpatient or outpatient setting. If all goes well, monoamniotic twins should be delivered from 33 weeks’ gestation as, at that timepoint, the risk of in utero death is larger than the risk of (non-respiratory) neonatal complications.