The proband’s clinical presentation, with onset of focal febrile status epilepticus associated with immunization followed by the emergence of refractory seizures and cognitive development delay, suggests that the proband suffered from epileptic encephalopathy associated with a mutation in SCN1A, a condition that is milder than expected in Dravet syndrome. This evidence concerns the gene SCN1A and encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.