A 45-year-old woman with adrenocorticotropin (ACTH)-independent hypercortisolism, diabetes mellitus, and hypertension had undergone left adrenalectomy for ACTH-independent Cushing's syndrome 20 years prior to this presentation. There was cushingoid appearance 1 year after surgery. However, Cushing's syndrome recurred; ACTH-independent Cushing's syndrome was diagnosed and abdominal computerized tomography showed a right adrenal tumor, which was removed. Histology revealed primary pigmented nodular adrenocortical disease (PPNAD). The patient had also undergone hysterectomy for uterine masses diagnosed as uterine myxoma. Right breast and neck skin masses were also found, both of which were removed and diagnosed as mammary myxoid fibroadenoma and cutaneous myxoma. She had a homozygotic twin sister who also had Cushing's syndrome and had undergone bilateral adrenalectomy 13 years previously with a pathologic diagnosis of PPNAD. The twin sister also had skin, breast, and uterine masses, all of which were resected. The pathologic results were the same as this patient's. According to the clinical presentations, histologic findings, and positive family history, familial PPNAD (Carney complex) was diagnosed.