Over the last decade, several entities within the spectrum of acute febrile encephalopathy (AFE) including acute disseminated encephalomyelitis, acute necrotizing encephalitis, fever-induced refractory epilepsy syndrome, clinically mild encephalopathy with reversible splenial lesion, and autoimmune encephalitis have been recognized, all of which now have reasonably well-established clinicoradiological diagnostic criteria and therapeutic options. This case series highlights the clinical profile and outcome of seven children with the lesser-known entity of AFE with seizures and white matter diffusion restriction lesions on magnetic resonance imaging. All children had a history of acute onset fever, altered sensorium, and refractory seizures requiring multiple antiepileptic drugs and presented in shock requiring multiple organ support. All had extensive, diffusion-restricted lesions involving white matter. The infectious trigger was identified as influenza A (H3N2) in case 1, dengue virus in cases 2 and 6, and influenza B in case 4. Five children survived, each of whom had significant neurological sequelae.