The most commonly recognized group of disorders are the long QT syndromes (LQTS), in their several genetic forms due to mutation in one of these ion channel genes: KCNQ1, KCNH2, KCNE1, KCNE2, KCNJ2, KCNJ5, SCN5A, SCN4B, ANK2, CAV3, AKAP9, SNTA1, CALM1, CALM2, and CACNA1C. We describe a family presenting with syncope, dysrhythmia including ventricular fibrillation, and sudden cardiac death, segregating with an autosomal dominant R858H variant in the CACN1AC gene. The gene discussed is CACNA1C; the disease is familial long QT syndrome.