In clinical practice, various diseases manifest as extrapyramidal symptoms. Diagnosing these disorders is crucial, as they are often accompanied by psychiatric symptoms and other systemic manifestations that affect prognosis, in addition to motor symptoms. This article provides an overview of Huntington's disease, neuroacanthocytosis, hereditary dystonia, and ADCY5-related dyskinesia, which are relatively prevalent in the Japanese clinical setting. The discussion centers on motor symptoms, therapeutic approaches, and underlying genetic causes.