The authors describe a unique presentation and long-term management of a rare craniovertebral abnormality in a patient presenting to their institution. This 10-year-old girl presented with right-sided facial pain and subjective dysesthesia of the chest wall without evidence of cervical myelopathy. She was found to have extensive cervicothoracic syringomyelia secondary to compression at the foramen magnum by hypertrophic occipital condyles. Posterior decompression and medial condylectomy was performed, with significant radiological and clinical improvement over the next 5 years of follow-up. The authors discuss the clinical pathophysiology and operative techniques used.