Tuberous sclerosis complex (TSC) is a rare autosomal dominant disease due to pathogenic variants in TSC1 or TSC2 genes. In the brain, TSC is associated with multiple cortical and subcortical malformations including tubers and abnormalities of radial neuronal migration. Approximately 80% of patients develop epilepsy in the first two years of life, most often focal seizures and infantile spasms. As with all seizure disorders, systemic illness and fever can trigger a seizure, and result in status epilepticus or even refractory status epilepticus. Infantile Hemiconvulsion-Hemiplegia and Epilepsy (IHHE) is considered a subcategory of new-onset refractory status epilepticus (NORSE) and presents with hemiclonic seizures in the setting of fever, unihemispheric brain imaging abnormality and hemiparesis. Here, we present an 18-month-old boy with TSC who developed IHHE. His extensive brain malformations and neuronal hyperexcitability in peri-tuberal tissue could have predisposed him to IHHE. In addition to these factors, we postulate that another prerequisite for IHHE is likely a genetic predisposition for an excessive inflammatory response that is yet to be elucidated.