Introduction: Vagus nerve stimulation (VNS) has been used in both adults and older children with varying success. Materials and methods: We retrospectively reviewed our experience with VNS in very young children (below 5 years old). The mean age at stimulator implantation was 20.5 months. Two patients were below 2 years old at implantation and two patients were below 1 year old at their initial surgery. The average follow up time for this group was 22 months. Results: Of the six patients (three males and three females) with long-term follow up, 83% had a significant decrease in the frequency of their seizure. Of these, two are seizure-free (33%), three are improved (50%), and one (17%) has had no change in seizure status at their most recent clinical examination. Age at implantation of the vagus nerve stimulator did not seem to correlate with patient success. In this group, atonic seizures were found to best respond to VNS with cessation of this type of seizure in two patients. No patients were made worse by the procedure and no morbidity was observed related to VNS. Conclusions: Based on our small patient cohort, it appears that VNS in very young children with life-threatening epilepsy can be efficacious. Larger groups and other institutional experiences are now needed to verify our findings. © Springer-Verlag 2006.