Twin-to-twin transfusion syndrome presents in the mid-trimester with gross discordance in amniotic fluid volume, and complicates 10-15 per cent of monochorionic twins. Recent studies suggest a primary vascular basis in which a paucity of the bidirectional superficial anastomoses normally found in monochorionic twins is unable to compensate for haemodynamic imbalance resulting from unidirectional transfusion along deeper arterio-venous anastomoses. It is associated with high rates of perinatal mortality from ruptured membranes, hydrops and growth restriction, and a significant morbidity from cardiac and neurological sequelae in particular. Serial aggressive amnioreduction is the current treatment of choice, with survival in around two thirds of cases. In the remaining third, with features suggesting a poor outcome, selective fetocide may have a role. Current attempts at vascular ablative therapies have been associated with inferior survival rates, but the long term therapeutic goal remains the identification and ablation of the shared chorionic vasculature.