Neuromyelitis optica spectrum disorder (NMOSD) is a multifocal central nervous system demyelinating disease, and is associated with NMO-immunoglobulin G or anti-astrocyte water channel aquaporin-4 antibody. We report a 74-year-old woman with NMOSD who presented choreoathetosis. She developed left upper limb weakness and sensory impairment below the left C2 level, and the lesion was located primarily in the gray matter from the lower brainstem to the C6 level. Her symptoms were ameliorated after steroid pulse therapy, but painful tonic spasms (PTS) occurred in the affected limb. After gabapentin (600 mg/day) was started for the PTS, choreoathetotic movement appeared in her left upper limb. We suggest that modification of the gamma aminobutyric acid receptor activity and the cervical gray matter lesion had a role in the development of choreoathetosis.