Our aim was to review the literature on umbilical cord coiling. Relevant articles in English published between 1966 and 2003 were retrieved by a Medline search and cross-referencing. The normal umbilical cord coiling index (UCI) is 0.17 (+/- 0.009) spirals completed per cm. Abnormal cord coiling, i.e. UCI <10th centile (<0.07) or >90th centile (>0.30) is associated with adverse pregnancy outcome. Hypocoiling of the cord is associated with increased incidence of fetal demise, intrapartum fetal heart rate decelerations, operative delivery for fetal distress, anatomic-karyotypic abnormalities and chorio-amnionitis. Hypercoiling of the cord is associated with increased incidence of fetal growth restriction, intrapartum fetal heart rate decelerations, vascular thrombosis and cord stenosis. It is not clear whether abnormal coiling is actually a cause of pathology, or merely one of the sequelae, or both. We discuss the theories involving the cause of cord coiling, and the consequences of the degree of cord coiling on blood flow through the umbilical vessels. In the future ultrasonographic evaluation of the umbilical cord and the UCI may become an integral part of fetal assessment in high-risk pregnancies.