BACKGROUND: Malignant vasovagal syncope (VVS) is a unique form of cardioinhibitory VVS, characterized by prolonged asystole. To deepen the understanding of this distinct type of VVS in children, this article reviews advancements in the potential pathogenesis, diagnostic approaches, clinical significance, and treatment controversies of malignant VVS in pediatric patients. DATA SOURCES: This article was developed by reviewing the literature and studies in databases including PubMed and Chinese Journal Full-text Database up to September 2024. Search terms included "malignant vasovagal syncope" AND "children" or "vasovagal syncope" AND "asystole" AND "children". RESULTS: To date, studies focused on malignant VVS in children remain limited. Theoretically, children with malignant VVS are at risk of physical or mental impairment due to this cardiac asystole, though no definite conclusions have been reached. The mechanism underlying the significant cardiac inhibition in malignant VVS remains unclear, and the treatment strategy of malignant VVS is debatable. CONCLUSIONS: The understanding of children with malignant VVS is insufficient. Further research is required to evaluate the clinical features and the pathogenesis of pediatric VVS with cardiac asystole and to establish effective management strategies for malignant VVS. Video Abstract.