Monoamniotic twin pregnancies are rare, but are associated with a high risk of perinatal death. The major complications are due to cord entanglement and twin twin transfusion. We were recently faced with the clinical problem of a monoamniotic twin pregnancy complicated by cord entanglement. Fortunately the outcome was favourable with the survival of both twins after delivery by elective Caesarean section at 32 weeks' gestation. Our case as well as some of the controversies in the management of this unpredictable condition are presented. Most authors seem to prefer abdominal delivery upon reaching fetal lung maturity in the case of known monoamniotic twins although this management is not validated by the available studies. Hospital admission for fetal monitoring upon discovery of a cord entanglement at a viable gestation is also advocated.