True knots of the umbilical cord (UC) are a rare occurrence and are reported in 0.4–1.2% of deliveries. The compression of true knot of the UC can cause obstruction of the fetal circulation, leading to intra‐uterine growth retardation or fetal death. Predisposing factors for the genesis of the true UC knot are numerous and include all the conditions, which lead to a relatively large uterine volume. This situation may predispose to free and excessive fetal movements. Although not all true knots lead to perinatal complications, they have been associated with adverse pregnancy outcomes, including fetal distress, fetal hypoxia, intra‐uterine growth restriction (IUGR), long‐term neurological damage, caesarean delivery and stillbirth. We present a rare case of operative delivery with vacuum in a multiparous woman at term of pregnancy with a double true knot of the UC. As in most cases, the diagnosis was made after delivery, as there were no fetal symptoms during pregnancy. Some authors assume that 3D power sonography may be useful in the diagnosis of true UC knots. However, 3D power Doppler cannot be considered as a definitive method. There are no specific prenatal indications to induce the physician to look for ultrasound signs suggestive of umbilical true knot. Some studies argue that cases of fetal death and fetal risk are directly related to the number of knots. We also support this thesis, even if further observational and retrospective studies are needed to demonstrate it.
