Recent increases in presentation of occipital deformities have presented the craniofacial team with new challenges in diagnosis and treatment. As presented in this article, they appear to have a functional abnormality of the suture that results in a localized growth deformity. The growth inhibition in severe cases can result in changes in the local bone and brain, along with the entire skull and cranial base. Histologic analysis supports the theory that this is true lambdoid synostosis. Surgical correction is indicated in moderate to severe cases, and the authors have presented their technique and new data on outcomes.