While the inactivating mutations in the PRKAR1A gene are responsible for Carney Complex (CNC), the gain-of-function mutations of this gene have been identified in patients with acrodysostosis, a heterogeneous group of rare skeletal dysplasia [37,38], suggesting the possibility of a direct involvement of the PKA-regulated pathway in regulating bone modeling and/or remodeling, and presumably being responsible for the development of both chondrodysplasia and osteochondromyxomas when deregulated. This evidence concerns the gene PRKAR1A and chondrodysplasia.