We report the case of a female adolescent diagnosed with LQT2, in whom the identification of a mutation p.(Ala614Val) (ClinVar ID: VCV000029777.14) in the KCNH2 gene, the persistence of cardiac events under beta blocker treatment, and an increased SCD risk, allowed the configuration of a high-risk profile aiding the decision to implant an ICD. The gene discussed is KCNH2; the disease is Schnyder corneal dystrophy.