Background: Leucine‐rich, glioma‐inactivated (LGI1) antibody encephalitis is a rare autoimmune encephalitis characterized by faciobrachial dystonic seizures (FBDS), temporal lobe epilepsy, prominent short‐term memory deficits and altered mental status. FBDS are very brief, frequent stereotypical dystonic seizures of the ipsilateral face, arms and legs. FBDS are often initially unilateral and evolve bilaterally or asynchronously. Case presentation: A 49‐year‐old woman developed frequent FBDS of her right side. Fluid attenuated inversion recovery imaging showed hyperintense lesions in the right striatum. She was treated with only anti‐epileptic agents. FBDS emerged on her left side soon after cessation of FBDS on her right side. LGI1 antibody was positive in the serum and cerebrospinal fluid. Steroid therapy was added on day 157, and thereafter, FBDS subsided completely, although cognitive impairment remained. Conclusions: A case of LGI1 antibody encephalitis with asymmetrical manifestation of FBDS after resolution of the first unilateral symptoms was described. This case suggests that LGI1 antibody encephalitis can affect each side of the brain asymmetrically and asynchronously. [ABSTRACT FROM AUTHOR]