Although these manifestations have been reported in neuromyelitis optica spectrum disorder (NMOSD), they are typically associated with cervical spinal cord lesions, and occurrence due to isolated medullary demyelination is extremely uncommon.<h4>Case report</h4>A 32-year-old woman with aquaporin-4-positive NMOSD developed pseudoathetotic movements with dystonic-appearing posturing of both hands following an isolated demyelinating lesion of the medulla with normal spinal cord imaging. Here, AQP4 is linked to neuromyelitis optica.