目的　总结偏侧惊厥-偏瘫-癫痫（HHE）综合征的临床特点及诊断。方法　回顾分析2017－2019年收治的3 例HHE综合征患儿的临床资料，并进行文献复习。结果　3例患儿均为女性，发病年龄分别为3岁、 1岁2个月和6岁11个 月。首次惊厥均伴有发热，且均发生癫痫持续状态， 2例出现右侧偏瘫、 1例出现左侧偏瘫。 1例患儿有肝功能、心肌酶异常。 3例患儿的头颅磁共振成像或头颅CT均有异常改变，且与临床症状相对应。 3例患儿均在偏瘫后随之出现反复癫痫发作， 均给予奥卡西平为主的抗癫痫药物治疗，癫痫均暂时完全控制。例1检测到染色体微阵列chr5q33.3q34处存在9.1Mb缺失， 包含GABRA1、GABRA2等大量功能基因，例2基因检测未发现异常，例3未行基因检测。结论　HHE综合征是儿童惊厥性 癫痫持续状态的罕见后果，早期正确处理惊厥持续状态可以有效减少HHE综合征发生。

　Objective　To summarize the clinical characteristics and diagnosis of hemiconvulsion -hemiplegia-epilepsy (HHE) syndrome. Methods　The clinical data of 3 children with HHE admitted in the Department of Pediatrics, the Affiliated Hospital of Qingdao University from 2017 to 2019 were retrospectively analyzed with related literatures reviewed. Results　All the 3 cases were female. The age of onset was 3 years old, 1 year and 2 months old, 6 years and 11 months old, respectively. All the first convulsions were accompanied by fever and convulsive status epilepticus. Right hemiplegia occurred in 2 cases and left hemiplegia in 1 case. Abnormal liver function and myocardial enzymes occurred in 1 case. Abnormal changes in cranial magnetic resonance imaging or cranial CT were observed in all 3 children which is in accordance with clinical symptoms. After hemiplegia occurred, 3 cases had recurrent epileptic seizures which were completely controlled temporarily after being treated with oxcarbazepinebased antiepileptic drugs. Gene detection was performed in case 1 and case 2. In case 1, 9.1Mb deletion in chr5q33.3q34 including a large number of functional genes such as GABRA1 and GABRA2 were detected by chromosome microarray. In case 2, no abnormality was found. Conclusion　HHE syndrome is a rare consequence of convulsive status epilepticus in children. The occurrence of HHE syndrome can be effectively reduced through further understanding of the syndrome and early appropriate treatment of status convulsion.