In children, inflammatory brain diseases (IBrainDs) are increasingly being recognized. Clinical, neuroimaging and laboratory features are overlapping, and an exact diagnosis can be significantly delayed. Novel antibodies have been discovered and should be included in the diagnostic evaluation. Specific neuroradiological tests such as conventional angiography or vessel wall enhancement can assist in supporting the diagnosis. Brain biopsy should be considered in children with IBrainD with unclear pathology. Treatment should be tailored to the underlying pathogenesis.