Twin reversed arterial perfusion (TRAP) sequence is a specific and severe complication of monochorionic multiple pregnancy, characterized by vascular anastomosis and partial or complete lack of cardiac development in one twin. Despite its rarity, interest in the international literature is rising, and we aimed to review its pathogenesis, prenatal diagnostic features and treatment options. Due to the parasitic hemodynamic dependence of the acardiac twin on the pump twin, the management of these pregnancies aims to maximize the pump twin's chances of survival. If treatment is needed, the best timing of intervention is still debated, although the latest studies encourage intervention in the first trimester of pregnancy. As for the technique of choice to interrupt the vascular supply to the acardiac twin, ultrasound-guided laser coagulation and radiofrequency ablation of the intrafetal vessels are usually the preferred approaches.