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.