© 2013 Springer Science+Business Media New York. All rights reserved. Clinical presentation of the Chiari I malformation varies with patient age, and children typically present with shorter symptom duration than adults. Symptoms and signs of Chiari I malformation in children are most often attributable to impairment of CSF flow, direct neural compression, and/or syringomyelia. Comparison of contemporary and older literature reveals a trend toward earlier diagnosis, prior to manifestation of significant neurologic morbidity. General availability and use of magnetic resonance imaging likely explains this dramatic shift. Occipital and/or cervical pain is the most common presentation, particularly if Valsalva-induced, followed by scoliosis, syringomyelia, and lower cranial nerve dysfunction. Headache not localized to the occipital/cervical region correlates poorly with Chiari I malformation. The natural history of asymptomatic children with Chiari I malformation (absent syringomyelia) appears benign. Surgical treatment should be reserved for patients with localized pain, syringomyelia, or cervicomedullary/cerebellar dysfunction.