Pathogenic variants in POLG are one of the most common causes of inherited mitochondrial disorders [5], and are associated with a wide spectrum of clinical manifestations ranging from early-onset disease with therapy- resistant epilepsy and liver failure, to juvenile and adult-onset disease with epilepsy, stroke-like episodes (SLEs), ataxia, and peripheral neuropathy, and to late-onset disease with progressive external ophthalmoplegia (PEO) [5–8]. Here, POLG is linked to epilepsy.