Background Influenza-associated neurological disease (IAND) is uncommon but can result in death or neurological morbidity in children. We aimed to describe the incidence, risk factors, and outcome of children with IAND from seasonal influenza in Australia. Methods We analyzed national, population-based, surveillance data for children aged ≤ 14 years with severe influenza and neurological involvement, over 11 Australian influenza seasons, 2008–2018, by the Australian Paediatric Surveillance Unit. Results There were 633 laboratory-confirmed cases of severe influenza reported. Of these, 165 (26%) had IAND. The average annual incidence for IAND was 3.39 per million children aged ≤ 14 years. Compared to cases without neurological complications, those with IAND were more likely to have a pre-existing neurological disease (odds ratio [OR] 3.03, P < .001), but most children with IAND did not (n = 135, 82%). Children with IAND were more likely to receive antivirals (OR 1.80, P = .002), require intensive care (OR 1.79, P = .001), require ventilation (OR 1.99; P = .001), and die (OR 2.83, P = .004). Conclusions IAND is a preventable cause of mortality, predominantly in otherwise well children. Incidence estimates validate previous sentinel site estimates from Australia. IAND accounted for a quarter of all severe influenza, is associated with intensive care unit admission, and accounted for half of all influenza deaths.