The case of a 41-year-old woman with recurrent cardiac myxomas and widespread lentiginosis is reported. The diagnosis of a Carney complex was established 7 and 25 years, respectively, after first manifestation of the cardiac myxomas in both the patient and her brother. This peculiar hereditary disease is commonly associated with multiple neoplasms and an endocrine overactivity, requiring a thorough examination of the patients and their relatives to detect additional typical manifestations.