A 29-year-old primigravida was referred at 25 weeks of gestation for evaluation of a growth discrepancy in a monochoronic diamniotic twin pregnancy. On ultrasound examination, the first fetus appeared to have grown normally, while biometry was consistently below the 5 th centile in the second fetus. In this small-for-gestational age (SGA) fetus, the umbilical artery pulsatility index (PI) was above the 95 th centile for gestation, and the middle cerebral artery PI was within the normal range. A diagnosis of selective early-onset intrauterine growth restriction (IUGR) was made. Color Doppler was used to image the ductus venosus (DV). The DV originated normally as a branchless straight vessel from the portal sinus 1,2 . However, instead of converging together with the hepatic veins into the subdiaphragmatic vestibulum, the DV drained separately into the inferior vena cava, approximately 1 cm below the right atrium (Figures 1 and 2). Pulsed wave Doppler demonstrated an abnormal pattern of flow with reversed A-wave velocities. Reversed flow in the DV is usually indicative of fetal compromise 3,4 . Albeit growth-restricted, the fetus did not demonstrate any other signs of severe fetal distress on umbilical and middle cerebral artery Doppler, biophysical profile score (BPS), or computerized cardiotocography (cCTG), and the pathological waveform in the DV was attributed to the abnormal anatomical arrangement. It was therefore decided not to use DV Doppler findings for timing the delivery. In the following weeks, all monitoring parameters remained within the normal ranges, except for a gradually increasing umbilical resistance in the SGA twin. At 32 + 5 weeks, reversed end-diastolic flow was demonstrated in the umbilical artery. Given the normal BPS and cCTG, a full course of steroids was given, and two male infants weighing 1730 g and 1420 g were delivered by elective Cesarean section at 33 + 3 weeks. On day 4, an abdominal ultrasound examination was performed on Twin 2, confirming normal liver vascular anatomy and the spontaneous closure of the DV. Both newborns developed severe respiratory distress syndrome and Staphylococcus epidermidis sepsis. Twin 1 recovered uneventfully, while Twin 2 died on day 20. Placental examination confirmed the diagnosis of a monochorionic diamniotic placenta, with velamentous insertion of the cord of Twin 2 and angiodystopias. The parents did not consent to a postmortem examination.DV Doppler is being used increasingly for fetal assessment in cases of early onset IUGR 4 , and its role is currently being investigated in a large randomized trial 5 . In the present case, a grossly abnormal blood flow pattern was detected 8 weeks before delivery. We attributed this finding to the anatomical variant of a DV draining into the inferior vena cava instead of the subdiaphragmatic vestibulum 2 , with the abnormal branching angle possibly affecting the transmission of atrial contraction waveforms 6 . Antenatally, the differential diagnosis included a portocaval shunt with agenesis of the DV, and an ...