Twin embolization syndrome (TES) is a rare complication of twin-twin transfusion after intrauterine demise of one of a pair of twins sharing the same placenta. In such cases, infarcts in the surviving co-twin can cause structural and functional changes, presumably due to embolization of debris, thrombus, thromboplastic material, and toxins from the dying twin's circulation into the circulation of the other twin. Sonographic, clinical, and pathologic findings in 3 new cases are reported, and 38 previously reported cases of TES are reviewed.Of the 41 cases, 34 (83%) had evidence of a destructive structural brain defect. In the 34 cases with brain defects, 23 (68%) had intracranial cystic changes. Microcephaly had developed in the surviving twin by the time of delivery in 7 (41%) of the 17 cases with brain defects and evidence of twin fetal demise before 22 weeks. Intestinal atresia was noted in 4 of 41 cases (10%), and renal dysplasia or renal cortical necrosis was observed in 11 cases (27%). In cases of intrauterine demise of a monochorionic twin, careful follow-up evaluation of the surviving twin should be performed.