True knots of the umbilical cord are complications that can result in obstetric disasters, including fetal asphyxia and eventual fetal death. This study reports on 13 patients with a true knot of the umbilical cord in a delivery population of 967 deliveries during a 1-year period. In this group, there was one second-trimester fetal demise, four cases of fetal distress during labor necessitating cesarean delivery, and eight cases with a true knot found incidentally at delivery. No abnormal sequelae were found for the 12 viable newborns. True knots of the umbilical cord can be incidental findings; however, a tightened knot is reported to be associated with a fourfold increased risk of fetal death. Prenatal fetal surveillance, including targeted sonographic examination and Doppler studies, may identify true knots of the umbilical cord. Identification of high-risk patients and clinical expertise in the management of these patients have not been established.