Published studies differ concerning the rate of anomalies occurring in the presence of a single umbilical artery and the significance of the single umbilical artery as an isolated sonographic finding. We assessed the frequency, nature, and sonographic detection of structural anomalies in fetuses with a single umbilical artery. We identified all cases in which prenatal sonography diagnosed a single umbilical artery. Cases were excluded if postnatal physical or pathologic examination demonstrated a three‐vessel cord, yielding a study population of 167 cases. For each case, we recorded the gestational age at diagnosis of single umbilical artery and the findings of the sonographic fetal anatomic survey. We recorded postnatal clinical and pathologic information when available. Gestational age at time of diagnosis ranged from 16.8 to 41.1 weeks (mean, 29.2 +/‐ 6.5 weeks). Twenty of the 167 fetuses (12%) were twins, and the remainder were singletons. Among 118 cases with postnatal information, 37 (31%) had structural abnormalities, often involving multiple organs. The most common organ systems involved were the heart (19 cases) and the gastrointestinal (14 cases) and central nervous systems (nine cases). Five of the anomalous fetuses had abnormal karyotypes. The sonographic survey was abnormal in 31 of the 37 anomalous fetuses (84%). Among 85 cases with apparently isolated single umbilical artery at sonography and known fetal outcome, six (7%) proved to be anomalous at birth. We had two sonographic false‐positive results (mild hydronephrosis, suspected skeletal dysplasia). In summary, approximately one third of fetuses with single umbilical artery have structural anomalies, most often cardiac. Even when the single umbilical artery is an apparently isolated sonographic finding, the likelihood that the neonate will prove to have structural anomalies is considerable (7% in our series).