Cushing's syndrome is reported in close to 80 infants under 1 year of age. Infants present with obesity, growth failure, and cushingoid facies. Some are virilized. Adrenal tumors account for the majority of cases. The second most common cause is an adrenal hyperplasia or dysplasia that is usually nodular and may be secondary to a mutation in the stimulatory guanine nucleotide-binding protein. Pituitary adenomas and ectopic ACTH production are exceedingly rare in this age group. An infant with nodular hyperplasia is presented and the clinical features, etiology, diagnosis, and treatment are discussed.