Pseudoxanthoma elasticum (PXE) is a genetic disorder that affects the skin, eyes, and cardiovascular system. It is caused by homozygous or compound heterozygous mutations in the ABCC6 gene, and recent research reveals that heterozygote carriers of the ABCC6 mutation can manifest the characteristic phenotypic abnormalities of PXE. In this case report, Tiger et al document a granulomatous dermatitis with PXE-like changes in a patient with cystic fibrosis. The gene responsible for cystic fibrosis is ABCC7, an ABC cassette transporter gene in the same family as ABCC6. The relationship between these disorders remains unclear, but greater awareness of this phenomenon may promote further investigation to elucidate its clinical and biological significance.