As the biological mechanisms through which potassium channels mutations cause complex developmental phenotypes are still poorly characterized, the study of KCNH1 localization and its functions related to primary cilia and the alterations introduced by mutations in ciliogenesis, cell cycle coordination, cilium morphology, and cilia signaling pathway could help elucidate the molecular mechanisms underlying neurological phenotypes and neurodevelopmental disorders not considered as classical ciliopathies, but for which a significant role of primary cilia is emerging. This evidence concerns the gene KCNA3 and neurodevelopmental disorder.