PURPOSE: Epilepsy with myoclonic atonic seizures (EMAtS) is a rare childhood-onset developmental and epileptic encephalopathy, often resistant to pharmacologic treatments and is associated with significant cognitive impairments. Early seizure control is crucial for optimal neurodevelopmental outcomes. This study describes seizure frequency and neurodevelopmental outcome following vagus nerve stimulation (VNS) implantation in children with EMAtS. METHODS: We conducted a retrospective review of pediatric patients with EMAtS who underwent VNS implantation to treat their refractory seizures from 2011 to May 2024. RESULTS: Six pediatric patients with EMAtS underwent VNS implantation and were followed for an average of 4.6 years post implantation. Six months post-VNS activation, significant improvement in seizure frequency or intensity was observed in five patients, with four achieving a mean seizure reduction of 76 %. At the last follow-up (mean of 8.1 years), three patients were seizure-free. Positive neuropsychological outcomes were present in four patients. CONCLUSION: These findings suggest that VNS can be an effective therapeutic option for pediatric patients with refractory EMAtS, leading to improvements in seizure control and neurodevelopmental outcomes.