Leucine-rich glioma-inactivated 1 (LGI1) encephalitis is a form of autoimmune encephalitis (AE) that presents with memory loss, faciobrachial dystonic seizures (FBDSs), disorientation, psychiatric symptoms, and hyponatremia. Diagnosis is based on clinical presentation, magnetic resonance imaging (MRI), serum or cerebrospinal fluid (CSF) antibody testing, and electroencephalography (EEG) findings. Most available studies on its clinical features and treatment are limited to case reports and series, highlighting the need for a comprehensive understanding and standardized treatment approach. Herein, we report a 61-year-old Saudi retiree with cognitive decline, recurrent right FBDS, generalized tonic-clonic seizures, and persistent hyponatremia who showed a significant improvement after rituximab therapy.