Purpose <p>Isolated foot drop is a rare clinical finding in paediatric patients and is most often attributed to peripheral neuropathies or radiculopathies. Its occurrence as the sole presenting symptom of Chiari I malformation with syringomyelia is exceptionally uncommon. We describe a case of a 4-year-old girl presenting with isolated foot drop due to a holocord syrinx and review previously published paediatric cases.</p> Methods <p>A case report was compiled from clinical presentation, imaging, neurophysiology, operative details, and follow-up. Additionally, a review of 13 published paediatric cases of foot drop associated with Chiari I malformation and syringomyelia was conducted, with emphasis on clinical features, surgical management, and outcomes.</p> Results <p>The patient presented with sudden-onset, painless right foot drop without trauma or systemic symptoms. MRI revealed Chiari I malformation with extensive syringomyelia. Nerve conduction studies and laboratory investigations excluded peripheral causes. Following delayed posterior fossa decompression and C1 laminectomy with duraplasty, full neurological recovery was observed within 8&#xa0;weeks. Review of the literature identified 13 paediatric cases; in 10, foot drop was the only presenting symptom. Most underwent decompression surgery, with the majority achieving complete recovery. Conservative management was rarely attempted, and outcomes were not well documented.</p> Conclusion <p>Isolated paediatric foot drop may be an atypical presentation of Chiari I malformation with syrinx and should prompt timely neuroimaging. Surgical decompression is associated with favourable outcomes, even after short delays, supporting urgent but not necessarily emergent intervention. Greater awareness of this unusual presentation may facilitate earlier diagnosis and optimal management.</p>