We communicate a case with the Carney triad (gastric leiomyosarcoma, pulmonary chondromatosis and extra-adrenal paraganglioma). It is, to our knowledge, the first case to be communicated in the Spanish scientific literature. We discuss some peculiar aspects of the debut and clinical evolution of this syndrome, together with its prognosis. We conclude that in clinical practice, the appearance in a young subject, specifically females, of multiple gastric myogenic tumors, should elicit the performance of further noninvasive procedures, needed to discard the diagnosis of the Carney triad.