Twin reversed arterial perfusion sequence (TRAP) resulting in an acardiac twin is a rare occurrence. Acardia requires the presence of arterial-arterial anastomosis, with retrograde perfusion of poorly oxygenated blood from the normal twin to the acardiac twin, venous-venous anastomosis carrying blood back from the acardiac to the normal twin, and circulatory failure of the acardiac twin. Although vascular anastomoses in cases of TRAP have been well described, there is little published literature on the microscopic changes in the placenta in TRAP sequence. We report a case of monochorionic monozygotic twins with an acardiac twin, large arterial-arterial anastomosis, and direct continuity of the umbilical cord veins. The placenta showed villous immaturity, striking villous calcifications, and extensive fetal thrombotic vasculopathy of the umbilical vein of the donor twin and the chorionic plate vessels. Thrombi in the umbilical vessels of the acardiac twin have been reported, and trombi in donor twin organs have been seen after fetal demise in utero. But to our knowledge there is no previously reported thrombi in the donor twin umbilical vessels or the chorionic plate veins. This may have implications not only as a possible factor in the etiology of TRAP, but also and more important as a possible risk factor for thrombotic events in the donor twin. Further studies with clinico-pathological correlation are needed to explore this issue.