Background: We sought to analyze clinical features and surgical results of 10 cases of cardiac myxomas in Carney complex (CNC)., Methods: Between January 2003 and December 2013, 10 patients with cardiac myxomas in CNC underwent surgical resection. Associated cardiac lesions included moderate and severe mitral regurgitation in two cases, and moderate tricuspid regurgitation in one case. Age, gender, the incidence of arterial embolism, the rate of multiple cardiac myxomas, and the recurrence rate after resections of cardiac myxoma were compared between isolated cardiac myxomas and cardiac myxomas in CNC., Results: The incidence of cardiac myxoma in CNC was 1.74% (10/574). There were no deaths following surgery. There was one late death due to cerebral embolism 40 months following a reoperation (10%). A significant difference was found in the age, the incidence of arterial embolism, the rate of multiple cardiac myxomas, and the recurrence rate after resection of cardiac myxoma between cardiac myxoma in CNC and isolated cardiac myxoma (P < 0.05). There was no significant difference in gender between cardiac myxoma in CNC and isolated cardiac myxoma (P > 0.05)., Conclusions: Complex myxomas in CNS present at an earlier age, are more prevalent in women than in men, are more often multicentric, with a higher rate of arterial embolism and a high recurrence rate after resection. Close follow-up for cardiac myxoma in CNC after surgery is necessary due to the high recurrence rate., (© 2019 Wiley Periodicals, Inc.)