<p>Recent studies have shown that a ketogenic diet containing medium-chain triglycerides (MCTs) has a synergistic effect with perampanel in modulating AMPA receptors. Both MCTs and perampanel have immunomodulatory properties. However, the use of the combination of MCTs and perampanel to treat status epilepticus caused by autoimmune encephalitis has never been investigated. After multiple antiseizure (benzodiazepines, levetiracetam, brivaracetam, lacosamide, ketamine, propofol, and isoflurane) and immunomodulatory treatments (glucocorticoid pulse (days 4–8), intravenous immunoglobulin (days 10–12), plasmapheresis (days 13–19), rituximab (days 22 and 34), cyclophosphamide (day 38)), failed to interrupt status epilepticus in a 33-year-old woman caused by paraneoplastic anti-N-methyl-D-aspartate (NMDA) receptor encephalitis, we initiated perampanel treatment (12&#xa0;mg/day), which significantly reduced ictal activity in the EEG. Status epilepticus was finally terminated when MCTs were added to the perampanel treatment regimen. Our clinical data may support previous in vitro studies demonstrating that combining perampanel with MCTs improves the neuroimmunomodulatory effect on AMPA receptors. MCTs may have a synergistic effect when added to perampanel to treat autoimmune status epilepticus. The possible cumulative effects of combination with other immune therapies in our case cannot be completely excluded.</p> Graphical Abstract <p></p>