Klippel-Feil syndrome in its most basic definition includes several anomalous conditions of the cervicomedullary junction and suboccipital region. Pediatric neurosurgeons are often involved in surgical palliation of this syndrome, without realizing how the accompanying anomalies may obfuscate management in the older child. A brief review of the embryology of the rhombencephalon helps to clarify the etiology of some of these symptoms which may cause confusion and, occasionally, inappropriate treatment. Illustrative cases will demonstrate some of these pitfalls. Appropriate early intervention, such as posterior fossa decompression, ventricular shunting, and fundal plication, may help to avoid needless morbidity. The advent of magnetic resonance scanning has helped to clarify the diagnosis and resulted in more appropriate treatment in these cases.