Biallelic loss‐of‐function (LOF) variants of ABCC6 cause pseudoxanthoma elasticum (PXE) (Le Saux et al., 2000; Ringpfeil et al., 2000), a slowly progressing disease (Favre et al., 2017), characterized by soft tissue calcification with dermatologic, ocular, and cardiovascular manifestations, including vascular calcification, intermittent claudication, and higher incidence of cardiovascular events (De Vilder et al., 2018; Larusso et al., 2010). Here, ABCC6 is linked to Pseudoxanthoma elasticum.