ABSTRACT Twin anemia–polycythemia sequence (TAPS) results from slow intertwin blood transfusion through minuscule placental vascular anastomoses and is characterized by large intertwin hemoglobin differences in the absence of amniotic fluid discordance. The optimal management of TAPS is not clear. We report a case of TAPS detected antenatally by Doppler ultrasound examination at 15 + 6 weeks' gestation. After counseling, the parents opted for expectant management. Regular Doppler measurements were performed and these remained fairly stable. An emergency Cesarean section was performed at 34 + 5 weeks following signs of fetal distress. The donor twin was severely anemic while the recipient twin had severe polycythemia–hyperviscosity syndrome. On day 1, the recipient developed respiratory insufficiency and subclinical status epilepticus. Magnetic resonance imaging showed a total loss of gray–white matter differentiation as a sign of severe diffuse cerebral ischemia and bilateral intra‐ and extra‐axial hemorrhages. There was almost complete lack of arterial and venous cerebral blood flow. On day 3 intensive care treatment was withdrawn in view of the severity of the brain injury. This case report demonstrates that TAPS may lead to severe cerebral injury and fatal outcome in the recipient twin, and highlights the importance of antenatal Doppler ultrasound monitoring and choice of management. Copyright © 2012 ISUOG. Published by John Wiley & Sons Ltd.