To investigate the clinical manifestations, electroencephalogram (EEG) results, imaging characteristics, PRRT2 gene mutation features, and treatment methods of paroxysmal kinesigenic dyskinesia (PKD). Methods: A retrospective analysis was conducted on the demographic characteristics, clinical manifestations, EEG, imaging, PRRT2 gene testing results, and medication treatments of 4 PKD patients. The characteristics of PKD were summarized according to the above results. Results: Among the 4 PKD patients, there were 3 males and 1 female, with an age of onset ranging from 11 to 20 years. The clinical symptoms all manifested as unilateral or bilateral limb twisting and spasms triggered by movement, lasting no longer than 1 minute, and the consciousness was clear during episodes. In the long-term video EEG examinations, 2 patients showed no abnormalities. PRRT2 gene testing was conducted on 3 patients, among which 1 patient tested positive for a PRRT2 c.649dupC heterozygous mutation. Low doses of antiepileptic drugs could effectively control the episodes. Con? clusion: PKD has unique clinical characteristics and is easily misdiagnosed as epilepsy. Long-term video EEG detection has significant diagnostic value for PKD. PRRT2 gene testing is recommended when PKD is combined with other episodic diseases or when there is a positive family history. Long-term regular use of low doses of antiepileptic drugs can significantly improve seizures.