A case of multinodular pigmented adrenocortical hyperplasia occurring in a child aged 4 years is reported. The disease suggested an autonomously functioning lesion with low plasma ACTH. At surgery both adrenals presented with brown or yellow patches on the surface corresponding to multiple nodules. Unilateral adrenalectomy was followed by complete recovery and normal ACTH response to IV lysine vasopressin with a follow-up of 3 years. The patient also presented mild spastic diplegia and retarded mental development of unexplained origin.