Silver-Russell syndrome (SRS) is a rare genetic disorder characterized by prenatal and postnatal growth restriction, asymmetry of limbs and face, and other clinical features, affecting 1 in 30,000 to 100,000 live births with abnormalities in imprinted genes on chromosomes 7 and 11. Neurological involvement is rare in SRS, with only a few reported cases of tethered cord and low conus medullaris.

This case report presents a 13-year-old male with known SRS who presented with lethargy and seizures. The magnetic resonance imaging (MRI) revealed a downward displacement of the cerebellar tonsils through the foramen magnum and into the upper cervical canal, which is consistent with a type-1 Chiari malformation. The report highlights the importance of comprehensive medical evaluation and appropriate follow-up care in patients with genetic disorders to identify associated medical conditions, such as Chiari malformation, and provide timely interventions. Additionally, based on the limited reports of SRS patients with Chiari malformation, it may be prudent for pediatricians to conduct a thorough evaluation for this condition before recommending cGH therapy to prevent potential neurological complications and optimize clinical outcomes for SRS patients.