The incidence of true knot of the umbilical cord is not only very low but it is often undiagnosed antenatally when present despite the availability of prenatal ultrasonography. When the true knot remains tight, it may impede the circulation of the fetus and may result to fetal death in utero especially in labor. We report a very uncommon case of true knot of the umbilical cord that ultimately led to a fetal demise. A 39‑year‑old booked G7 P5+1 with four living children, who booked for antenatal care at a gestational age (GA) of 17 weeks. The fundal height was compatible with the GA throughout pregnancy. An ultrasound done at a GA of 36 weeks showed normal findings. She had onset of labor at a GA of 40 weeks and 5 days following cervical ripening with two courses of 50 μg misoprostol inserted at the posterior fornix 6 h apart. The fetal heart tones were monitored using Doppler sonicaid. They remained normal throughout labor that lasted 13 h. She subsequently had spontaneous vertex delivery with poor Apgar score. The baby died immediately after birth. The umbilical cord was more than double the normal length and a true knot of the umbilical cord was diagnosed after delivery of the baby. We have reported a case of true umbilical cord knot in Nnewi, South‑East Nigeria. Although this entity can be diagnosed antenatally, current experience is limited to incidental observation and consequent fetal demise.