Umbilical vein varix is an uncommon vascular anomaly. The varix may be the initial presentation of an underlying congenital portosystemic shunt adversely affecting the fetus as we demonstrate in this case. To be able to label a varix as an isolated finding necessitates a thorough evaluation of the fetal venous system as well. Serial ultrasound of the fetus can help in detecting changes in the varix, thrombosis, growth restriction, as well as heart failure if there is another underlying venous anomaly. Early postnatal imaging is essential to exclude other associations to optimize the neonatal outcome. The prognosis is good if it is an isolated finding.
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