Carney Complex is a multiple endocrine neoplasia syndrome characterized by heart, endocrine, skin, and neural tumors, as well as a variety of pigmented lesions of the skin and mucosal surfaces. The disorder may involve multiple endocrine glands, in particular thyroid and adrenal cortex. Carney Complex may include Cushing syndrome and micronodular adrenocortical hyperplasia, acromegaly, breast fibroadenoma, thyroid gland abnormalities and tumors, schwannomas, and/or osteochondromyxomas. Pigmented skin nevi and lentigines occur over the entire body, including lips and conjunctiva. Lesions can be brown-black, blue, or freckles. Myxoid tumors of many types may develop on any skin surface, including eyelids. Myxomas of the heart, especially atrial myxomas, are not uncommon. Carney Complex is due to mutations in the PRKAR1A gene on chromosome 17q22-q24, coding for the type 1-alpha regulatory subunit of protein kinase A. Carney Complex overlaps with Cushing disease, Primary Pigmented Nodular Adrenocortical Disease (PPNAD) and other adrenal hyperplasias, which in some patients have been associated with mutations in the PDE11A gene involved in cAMP signaling.