We report the NM_198056.2 c.2750 T>G p.(L917R) heterozygous variant in the SCN5A gene, together with its segregation in a large family to be associated with BrS, symptomatic palpitations, presyncope, conduction delay, and SCD. The pathogenicity of this variant is supported by its rarity in the general population, multiple pathogenic in silico predictions, and segregation with arrhythmic disease in this family. This variant was previously reported in association with BrS [7]; however, specific clinical information was not provided. The gene discussed is SCN5A; the disease is Schnyder corneal dystrophy.