BACKGROUND: Both the diagnosis and treatment regimens for the Chiari I malformation (CIM) are varied and controversial. The present paper analyzes the literature regarding this form of hindbrain herniation in regard to definition, anatomy, pathobiology, symptoms, findings, treatment, and outcomes. DISCUSSIONS: Appropriate literature germane to the CIM is reviewed and discussed. There is variation in the reported anatomy, outcome, and treatment for children with CIM. Based on the literature, most patients have preoperative symptoms or findings (e.g., syringomyelia) improve no matter what surgical technique is utilized. However, standardized treatment paradigms based on randomized controlled studies are still necessary to elucidate the optimal selection and treatment criteria.