Adrenal cystic lesions are rare and may be associated with both complete and incomplete Beckwith syndrome (BWS). Because the adrenal gland often houses malignant lesions, differentiation between benign and malignant lesions of the gland, although usually difficult, is very necessary from the point of view of management. Here we present our experience in a case of incomplete BWS with adrenal cystic lesion and review of the literature.