Few case studies of atypical presentation of pediatric Rasmussen's encephalitis (PRE) have been reported, but none of them described the emergence of super-refractory status epilepticus. Despite substantial advances in identifying the underlying routes, triggers, and etiology, the prognosis for Rasmussen's encephalitis (RE) patients remains uncertain. Epileptic spasms with delayed onset in RE patients are common but not drug-resistant epilepsy. In this article, we aim to describe an atypical presentation of PRE which was complicated by super-refractory status epilepticus.