Multiple pregnancies, the majority of which are twins, are at substantially higher risk of fetal morbidity and mortality when compared with singleton pregnancies. Single fetal demise occurs in up to 6.2% of all twin pregnancies. It may cause considerable risk for the co-twin including increased risk of fetal loss, premature delivery, neurovascular injury and end-organ damage. In this review we seek to summarise the most contemporary literature on the aetiology of single twin demise, the pathophysiology of injury to the surviving twin and the evidence for current management strategies.