Congenital anomalies of the umbilical vein are rare. We describe an aberrant course of the umbilical vein discovered by identifying an unusual umbilical venous catheter course on abdominal radiography in a patient with Cornelia de Lange syndrome. The umbilical vein bypassed the liver to insert directly into a right pelvic vein. Use of the lateral abdominal radiograph and sonography were helpful in determining the catheter location after identifying the unusual course of the catheter on the frontal radiograph.