Recently, Verdura et al. (2020) identified a homozygous pathogenic KCNA1 variant (p.Val368Leu), positioned in the S5–S6 linker, in a patient presenting with seizures and neurodevelopmental disorders in addition to EA1 symptoms, which was the first to be reported to act in a recessive mode of inheritance in KCNA1. The sibling described herein experienced typical EA1 symptoms including episodic ataxia and constant myokymia and unusual phenotypes such as seizures and learning difficulties. This evidence concerns the gene KCNA1 and neurodevelopmental disorder.