Lipomyelomeningocele represents a rare but complex neurological disorder that may present with neurological deterioration secondary to an inherent tethered spinal cord. Radiological testing is beneficial in determining the morphology of the malformation. Specialized testing such as urodynamic studies and neurophysiological testing may be beneficial in assessing for neurological dysfunction secondary to the lipomyelomeningocele. Early surgical intervention may be beneficial in preventing further neurological decline.