A 23-year-old male patient revealed hypercortisolism with stigmata of Cushing's syndrome, and post-operative pathological examination demonstrated primary adrenal nodular dysplasia. Because of the presence of cardiac myxomas and skin pigmentation, the diagnosis of Carney's complex was given. After the control of hypercortisolism by adrenalectomy, the patient experienced iridocyclitis and bilateral hilar lymphadenopathy with elevated levels of serum angiotensin-converting enzyme and lysozyme, all of which indicated the presence of sarcoidosis. Despite the numerous recent descriptions concerning Carney's complex, an association with sarcoidosis has not yet been documented. Moreover, the sequential occurrence of sarcoidosis after adrenalectomy suggests an etiological link between these two rare disorders.