Background: Childhood primary angiitis of the central nervous system (cPACNS) is a rare inflammatory disease of brain vessels. The small vessel subtype is diagnosed on brain biopsy and often presents with cognitive and behavioural changes, headaches and seizures. However, there are few reported cases of super-refractory status epilepticus. Methods: We present a case of small vessel cPACNS complicated by super-refractory status epilepticus and review the literature. Results: Our patient is a previously healthy 11-year-old boy who presented with new-onset seizures and encephalopathy in the context of fever. He developed super-refractory status epilepticus, requiring burst suppression for four weeks with various IV infusions. During this time, he was on the ketogenic diet and tried eight anti-seizure medications. Extensive investigations included brain biopsy confirming small vessel cPACNS. He was treated with IV methylprednisolone, oral steroids, IVIG, and cyclophosphamide. After prolonged rehabilitation, he recovered almost completely and has a normal neurological examination with no epileptiform activity on EEG. Conclusions: Small vessel cPACNS should be considered in the differential diagnosis of super-refractory status epilepticus. Despite being in SE for four weeks, symptomatic management of seizures and immunosuppression to treat the underlying pathology resulted in favourable neurological outcomes. This is one of the longest cases of SE in small vessel cPACNS in the literature.