Channelopathies comprise a spectrum of disorders in which ion channels and acetylcholine receptors are affected, although they could also be divided by the alteration of voltage-dependent or ligand/gatable channels to broaden their presentation. These alterations give rise to clinical manifestations depending on the affected channel subgroup, with epileptic encephalopathy being the most common presentation, in addition to ataxia, migraine, neurodevelopmental disorders, among others. The relevance of cerebral channelopathies in the pathogenesis of epilepsy and the diagnostic challenge related to their phenotypic heterogeneity have led to the recognition of new genes and genetic variants.