The discovery of PDE11A variants in patients with Carney complex (63) and genomic duplication of the locus of PRKACB (encoding the catalytic C-beta subunit of PKA) in a patient with Carney complex but without evidence of Cushing syndrome support the existence of versatile genomic alterations causing PKA dysregulation (101). The gene discussed is PDE11A; the disease is Carney complex.