The authors report a case of Carney's complex (the complex of myxomas, spotty pigmentation, and endocrine overactivity) in a 32-year-old white man. Psammomatous melanotic schwannoma has recently been included as a component of this disorder. The complex was recognized after a psammomatous melanotic schwannoma was resected from the sacral canal. Subsequent echocardiography revealed ventricular cardiac myxomata. Resection of these lesions was successfully performed. Review of previous history and pathologic material further supported the diagnosis. The authors conclude that recognition of each component as part of the syndrome is of crucial importance, because it may prevent a fatal outcome. A family screening program should be performed in each case.

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