The hair collar sign has been described as a marker of cranial dysraphism, including encephaloceles, meningoceles, and heterotropic brain tissue. This report describes a male born with a hair collar sign who subsequently was found to have a Klippel-Feil anomaly, diastematomyelia, multiple segmental spine and rib anomalies, and a translocation between chromosomes 1 and 4; t(1;4) (q44;q10). While not previously documented, spine abnormalities may be an associated abnormality with the hair collar sign. Physicians should consider radiographic evaluation of the spine in all newborns with a hair collar sign, as well as genetic screening.