Protection of telomeres 1 (POT1) is a component of the shelterin complex that has a critical role in regulating telomere length.1 2 Germline pathogenic variants (GPVs), including likely pathogenic (LP) variants, in POT1 have been associated with a variety of tumours resulting in the recognition of POT1-tumour predisposition syndrome (POT1-TPDS). Here, POT1 is linked to neoplasm.