Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), more commonly known as Hashimoto’s encephalopathy, is a rare, poorly understood, and probably under-diagnosed disorder. SREAT is characterized by a progressive cognitive decline, with neuropsychiatric features and possible seizures, myoclonus, and tremors in association with the presence of anti-thyroid, anti-thyroid peroxidase (TPO) and/or anti-thyroglobulin (TG) antibodies. Importantly, alternative explanations for encephalopathy must be excluded. There are no specific clinical, radiographic, or EEG abnormalities. MRI brain and CSF analysis can be normal. Antithyroid antibodies, either in serum or CSF, provide the key diagnostic clue but are not clearly pathogenic. The hallmark of SREAT is an excellent therapeutic response to steroids.