BACKGROUND AND PURPOSE: Patients with epilepsy are significantly more likely to die prematurely than the general population, with causes ranging from associated comorbidities to sudden unexpected death in epilepsy (SUDEP). The aim was to estimate the UK and Ireland incidence of childhood epilepsy deaths and to describe case demographics and clinical characteristics. METHODS: This was a prospective, population-based surveillance study using established active surveillance methodology designed by the British Paediatric Surveillance Unit. RESULTS: Eighty-eight confirmed cases were reported with an overall annual incidence of 0.65 per 100�000 children aged <16�years (95% confidence interval 0.52-0.81). More cases were male (65%) and cases fell across all age groups, with more deaths reported in older children. Twenty-five per cent of deaths were epilepsy-related (including SUDEP); 75% of deaths were non-epilepsy-related. SUDEP was the most common cause of seizure-related deaths, accounting for 13 out of 17 children (76%). An underlying epilepsy syndrome was present in 36% of deaths, and 88% had global developmental delay. In addition, 90% of the children had comorbid conditions in addition to epilepsy. CONCLUSIONS: In this study, it has been demonstrated that death in children diagnosed with epilepsy occurs mainly in 'complicated epilepsy' secondary to factors associated with neurodisability, consolidating previous data. SUDEP is also a significant cause of paediatric epilepsy mortality that needs further attention. There is a clear need to better understand and reduce the number of epilepsy deaths in children in the UK, and national surveillance of SUDEP is warranted to better understand this entity in paediatric populations.