The incidence of spinal injuries is increased in people with epilepsy although compressive thoracic myelopathy has not been reported. We describe a 15-year-old girl with SCN1A mutation (Dravet syndrome), refractory generalized tonic-clonic seizures, and prior posterior instrumentation and fusion for scoliosis, who presented with progressive lower extremity weakness. Junctional kyphosis with disc herniation and spinal cord compression directly rostral to the instrumentation was apparent on imaging. On history, the patient had suffered a particularly severe convulsive seizure just before developing symptoms. Surgical decompression and stabilization led to a complete neurologic recovery. This unusual presentation of myelopathy illustrates the need to consider this complication in patients with epilepsy and spinal instrumentation.