True umbilical cord knot is one of the umbilical cord abnormalities that can affect the pregnancy outcome. The existence of a true umbilical cord knot is associated with an increased incidence of fetal morbidity and mortality. Although it is possible to view the umbilical cord knot by imaging methods, for now prenatal diagnosis of a true knot is a random finding.The aim of this study was to determine the frequency of umbilical cord knot and to present the outcome of pregnancies with true umbilical cord knot. The analysis was performed retrospectively at the Department of Obstetrics and Gynecology of the University Hospital ˝Sveti Duh˝. The subjects were mothers who gave birth in the period from the beginning of 2005 to the end of 2017. Their newborns were also included in the study. In the researched group there were 267 mothers and newborns with the diagnosis of true umbilical cord knot. The control group included all mothers who had given birth to one child in the given period of time (n=39345). Stillbirth, mode of delivery – cesarian section or vaginal delivery, fetal asphyxia, and newborn's sex were analyzed for both groups. The data were collected from thehospital birth records and informatic system. The incidence of true umbilical cord knot was 0.67%. Comparison of the researched and control group did not establish a statistically significant difference in the mode of delivery (p=0.067), presence of fetal asphyxia (p=0.95). There was a statistically significant differencein the newborn's sex (p< 0.0001), and in the mortality (p< 0.0001) between the investigated and control group. The risk of intrauterine death is 15 times higher when a true knot is present. The existence of a true knot itself does not interfere with the blood flowthrough the umbilicalcord since there is no greater frequency of intrapartal fetal asphyxia when a true knot is present. However, the fact that there is a 15 times higher chance of intrauterine fetal death with a knotted cord is impressive. When and how a true umbilical cord knot becomes a threat to the life of the child is not yet established, and because of the higher risk of fetal death the only logical question is what to do if prenatal imaging methods determine the existence of a true knot.