Autoimmune anti-GABAB receptor encephalitis is a rare form of limbic en-cephalitis that typically has an initial neuropsychiatric presentation followed by se-qualae. Here, we present a case of anti-GABAB receptor encephalitis confirmed by cerebrospinal fluid testing and a corresponding left temporal lobe lesion detected by brain magnetic resonance imaging (MRI). Longitudinal evidence from clinical ob-servation, brain MRIs, and the ascending trajectory of his cognitive performance confirmed that the repeated high-dose corticosteroid pulse therapy effectively halted the patient’s disease progression with favorable responsiveness.