Congenital electrical and structural cardiomyopathies are rare and associated with an increased risk for syncope and sudden cardiac death in the young. Due to the young age of the patients and the limited data available, risk stratification and especially ICD therapy are challenging. In this young patient collective, ICD therapy is associated with a high complication rate, which does not justify unreserved primary prophylactic ICD implantation. The aim of this review is to elucidate risk stratification and ICD therapy of various electrical and structural cardiomyopathies.