Show a 14-year-old female patient treated at the William Soler Pediatric Cardiology Centre by fatigue. In complementary, she presented anemia and elevated ESR. Two-dimensional transthoracic echocardiography showed two tumors, one in the right atrium and the second in the left atrium. Removal is performed by right atrial transseptal approach using lung machine. Histopathological study showed that both tumors were myxomas. The patient had other tumors in skin, in external ear canal who were reported, as myxoma by biopsies performed. She was diagnosed as Carney syndrome.Five years later, recurrence myxoma in the right atrium that was removed. Eleven years after the first operation and six of the second, she is asymptomatic and free of tumors. Key words: intracardiac tumors, Carney syndrome.