In this article, we review the: (1) basic information about the CaV2.1 channel heteromultimer; (2) two relatively well-characterized diseases caused by mutation of the CaV2.1 α1A subunit—EA2 and FHM1; (3) the emerging full spectrum of CaV2.1 α1A channelopathies; and (4) the potential that the zebrafish model holds for understanding disease mechanisms and discovering potential therapeutics. Here, CACNA1A is linked to channelopathy.