A healthy woman, gravida 1, para 1, spontaneously delivered a stillborn at 40 weeks of gestation, presenting with a tight umbilical cord knot. Fetopathological examination confirmed the main but not exclusive role of the knot in this intra-uterine fetal death (IUFD). Knot of the umbilical cord is a rare occurrence, which is usually an incidental finding at delivery. However, it is associated with an increase in perinatal mortality. Only a complete fetopathological examination can state causality between knot and IUFD, exclude another fetal or placental cause of death and consequently reassure the parents for the prognosis of another pregnancy.
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