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We review the available literature to determinate the characteristics and the most appropriate management for this syndrome. The trend is surgical treatment of gastric GIST and paraganglioma, preferred conservative treatment in pulmonary chondroma. It is under discussion whether gastric tumors Carney syndrome is considered GIST, because the Carney triad lesions in stomach are different clinically, pathologically, and etiologically from GIST of stomach. Carney's triad is a low prevalence entity, with a limited number of