An umbilical vein varix is defined as the dilatation of the umbilical vein. It can develop inside the foetal abdomen (fetal intra-abdominal umbilical vein varix) or in the amniotic portion of the umbilical cord (intra-amniotic or extraabdominal). It can be associated with chromosomal abnormalities. An intra-amniotic umbilical vein varix is a rare abnormality. Its major complications, which can be fatal, include rupture, thrombosis, and compression and kinking of the umbilical cord. We describe the case of umbilical vein varix rupture that led to a IUFD of a male fetus at the 40th week of gestation.