ATP1A3 and Dystonia: Patients mainly present with paroxysmal hemiplegia, dystonia, abnormal eye movement, dyspnea, and other autonomic neurological phenomena.<h4>Patient concerns</h4>Here, we described a patient who initially presented with abnormal eye movements, followed by the subsequent development of seizures and alternating hemiplegia.<h4>Diagnoses</h4>Whole exome sequencing identified a heterozygous variant in the ATP1A3 gene: c.2443G > A (p.Glu815Lys).