Umbilical vein varices are rare umbilical cord anomalies that typically occur intra-abdominally. Extra-abdominal umbilical vein varices are exceedingly rare and usually diagnosed postnatally on gross pathologic examination. Umbilical vein varices have been associated with increased risk of fetal anemia, cardiac abnormalities, and intrauterine fetal demise. This case report discusses a patient who presented with a massive extra-abdominal umbilical vein varix, whose infant was ultimately delivered due to fetal distress and died in the neonatal period. This report also discusses associated fetal conditions and guidelines for antenatal testing and surveillance of known umbilical vein varices.