<h4>Background</h4>Pseudoxanthoma elasticum (PXE) is a rare disease resulting from ABCC6 gene mutations; it causes elastic fiber calcification with unclear arterial stenosis mechanisms.<h4>Case summary</h4>A 60-year-old man with PXE developed multivessel coronary stenosis despite low atherosclerotic risk. Here, ABCC6 is linked to Pseudoxanthoma elasticum.