A five-month-old male presented with an incidentally found low-lying conus medullaris on ultrasound and subsequent MRI demonstrating its position at L4. Pre-operative examination findings included mild, global hypotonia and a coccygeal dimple without bladder or bowel abnormalities or spasticity. The patient underwent spinal cord untethering with a section of filum terminale and was discharged without complication following his procedure. Follow-up at one year revealed continued baseline hypotonia without further neurosurgical needs. This is the first reported case of tethered cord syndrome described in a patient with Pallister-Killian syndrome managed successfully with neurosurgical intervention.
