The Carney complex (CNC), due to the germline inactivating mutation of PRKAR1A, is a familial tumor predisposition syndrome associated with growth-hormone (GH)-secreting pituitary adenoma, testicular Sertoli cell tumors, thyroid tumors and extra-endocrine manifestation such as myxomas, pigmented skin lesions, schwannomas, breast and bone tumors [21]. The gene discussed is GH1; the disease is schwannoma.