Seizures and epilepsy are the most common manifestations of all the evolutionary stages of NCC (78.8%, 95% CI: 65.1%-89.7%) . The seizure presentation can be an isolated seizure, seizures in cluster, and convulsive status epilepticus (CSE) [2,3]. CSE is an extremely rare presentation of all the evolutionary stages of NCC [2–5]. Extremely rarely CSE due to NCC may evolve into super refractory SE . However because of its rarity, CSE due to NCC has not been well characterized, hence this study.