The authors of this brief case report describe a previously unreported developmental variant of the craniovertebral junction. A 10-year-old girl who presented with cervical myelopathy was found to have cervicomedullary compression by hypertrophic occipital condyles. Decompression was achieved via a midline dorsal approach with no complications. The embryology of this area is briefly reviewed to attempt to explain the origin of this anomaly.