True knots are rare cord abnormalities but they are leading cause of cord accidents. Single umbilical artery and long umbilical cords exhibit a tendency to form true knots and nuchal cords. We aimed to show the association between single umbilical artery and other umbilical cord abnormalities which may lead to fetal compromise as in this case. We presented a pregnant women with intrauterine fetal demise associated with isolated single umbilical artery and other cord abnormalities. After delivery, the length of umbilical cord was noted to be 167 cm with four true knots on it. Those findings once again emphasize that true knots are serious risk factors for fetal death and could be associated with single umbilical artery and long umbilical cord. Therefore once single umbilical artery is detected on detailed sonography, examination must be extended to rule out other cord abnormalities and avoid cord accidents.