Hashimoto encephalopathy (HE) is a neuropsychiatric syndrome associated with positive thyroid antibodies (Ab). Its pathophysiology is still in debate and pediatric cases are considered rare. We present a case of an 11-year-old girl with new-onset refractory status epilepticus (NORSE) who presented a good initial response to corticosteroids but then required a second line of treatment with mycophenolate. In children presenting with NORSE of suspected autoimmune origin and no identification of autoimmune encephalitis traditional Ab, HE must be considered.