Abstract: Objective： To analyze the characteristics of antiepileptic drug hypersensitivity syndrome (AHS) and enhance medical staff′s awareness of this disease. Methods： Retrospectively analysis the clinical data of a clinically diagnosed AHS child. Results： The child was given sodium valproate oral solution 6 mL/time， 2 times/day. Phenobarbital tablets 45 mg/time， 2 times/day. Topiramate tablets 31.25 mg/time， 2 times/day. Due to autoimmune encephalitis and status epilepticus. One month later， the patient occurred persistent high fever， severe maculopapular rash， herpes， accompanied by itching， skin exfoliation， and respiratory system， kidney， myocardial damage， and diagnosed as AHS. After corresponding treatment， the patient′s condition improved. Conclusion： AHS is an idiosyncratic reaction，the pathogenesis is still unclear.Medication history， clinical manifestations， laboratory examinations are important basis for the differential diagnosis.Early identification， diagnosis，timely discontinuing of the suspected drugs and treatment are the keys to avoid fatal damage.