Abstract
Fibrous dysplasia is a benign bone disease characterized by the replacement of normal bone tissue with fibrous tissue, resulting in irregular bone structure. Cases of craniofacial fibrous dysplasia in children associated with Chiari type I malformation and syringomyelia are extremely rare. This case illustrates the complex clinical manifestations of craniofacial fibrous dysplasia along with Chiari type I malformation and syringomyelia, in which surgical intervention significantly improved the prognosis, and follow-up revealed near-complete resolution of the syringomyelia. It offers valuable insights for managing similar cases in the future.