Two probands had a diagnosis of cardiomyopathy, with woolly hair and keratoderma (OMIM 605676) and were compound heterozygous, each carrying a DSPtv (p.Arg2229Serfs*32 or p.Tyr28Alafs*66) and a DSP splice site variant (the same c.273+5G>A in both). Here, DSP is linked to Palmoplantar keratoderma.