The mutation in the cAMP-dependent protein kinase A (PKA) regulatory (R) subunit 1 (PRKAR1A) gene on loci 24.1–24.3 of the long arm of chromosome 17 has been identified as a cause of CNC.[5] More than 1000 CNC patients have been reported, of whom approximately 70% are familial cases. This evidence concerns the gene PRKAR1A and Carney complex.