Twin reversed arterial perfusion sequence, a severe and unique complication of monochorionic multiple pregnancy, is characterized by vascular anastomosis and abnormal or absent cardiac development in the twins. This article reviewed its pathogenesis, prenatal ultrasound diagnosis, and management. The pump twin’s chances for survival can be maximized by proper management. The optimal timing of the interventions remains a debate, although the latest studies encourage early intervention, i.e., in the first trimester. The most preferred approach is to interrupt the vascular supply to the acardius, such as through ultrasound-guided laser coagulation and radiofrequency ablation of the intrafetal vessels.