Encephalitis is a complex neurological syndrome caused by inflammation of the brain that occurs with highest incidence in children. It is challenging to diagnose and manage due to the variety of aetiologies and non-specific clinical presentations. We discuss the recent progress in clinical case definitions; review recent, large, prospective epidemiological studies; and describe aetiologies. We emphasise infectious causes relevant to children in Australasia but also consider emerging immune-mediated syndromes responsive to immune therapies. We identify priorities for future research in children, given the potential for climate change and international travel to influence the emergence of infectious agents in our region.