We reported a case of adrenal cytomegaly associated with Riedel's lobe of the liver, hepatonephromegaly, hyperplasia of islet cells of the pancrease and hypoglycemia. This case was analogous to the Beckwith syndrome. On the etiology of this phenomenon many hypotheses are contended. We thought the association of congenital anomalies with this phenomenon is important to the pathogenesis of this phenomenon.