Ultrarare inherited arrhythmia syndromes are increasingly diagnosed as a result of increased awareness as well as increased availability and reduced cost of genetic testing. Yet by definition, their rarity and heterogeneous expression make development of evidence-based management strategies more challenging, typically employing strategies garnered from similar genetic cardiac disorders. For the most part, reliance on anecdotal experiences, expert opinion, and small retrospective cohort studies is the only means to diagnose and to treat these patients. Here we review the management of specific ultrarare inherited arrhythmic syndromes together with the genetic and molecular basis, which will become increasingly important with the development of targeted therapies to correct the biologic basis of these disorders.