Abstract: Objective　To investigate the characteristics of ictal and interictal electroencephalogram (EEG) and clinical manifestation in children with startle epilepsy. Methods　The age of onset, inducing factors, the types of attacks, EEG features, cognitive function, treatment and prognosis were retrospectively analyzed in 8 cases of children with startle epilepsy from June 6, 2012 to March 2016. Results　In 8 cases, 3 cases were male and 5 were female. The onset age was from 2.3 to 8.1 years old. The forms of onset were varied from generalized (tonic, myoclonic, atonic) to partial seizures (the asymmetry of posture rigidity, spasm). The most common ictal EEG finding was a diffuse electrodecremental pattern (5 cases), and the interictal EEG showed a large number of multifocal, generalized spines, slow waves and multiple spinous waves. There was one case with no history of brain injury while the other 7 cases had a history of brain injury. There were 7 cases with imaging abnormality, and the lesions of the frontal, parietal and temporal regions were indicated with a partial cerebral softening and brain atrophy. In 7 cases, all children had abnormal mental and motor development, and 1 case had normal cognitive function. The 7 cases with shock epilepsy showed no obvious response to the treatment of multiple antiepileptic drugs, and 1 case had no clinical onset after 2 months of treatment with VPA. Conclusions　Startle epilepsy is mostly symptomatic, and few are non-symptomatic. The former had history of brain structure abnormalities, certain degree of motor retardation and mental disability, and no clinical response to antiepileptic drug therapy. The latter had basically normal cognitive function, and antiepileptic drug VPA treatment is effective. The degree of interictal epileptic was not an indicator of cognitive impairment and prognosis in children with startle epilepsy.