As with adults, the diagnosis of Cushing's disease in childhood can be difficult. The clinical presentation in children is distinctive in terms of the effects on growth and pubertal development. The biochemical diagnosis can usually be made with standard dexamethasone suppression testing, similar to such testing in adults. The clinical course of Cushing's disease in children tends to be more aggressive than in adults, as evidenced by higher rates of Nelson's syndrome after bilateral adrenalectomy and higher rates of recurrence after removal of corticotrope adenomas. TSS is well tolerated in children and has fewer side effects than other modalities. Currently, pituitary irradiation is best reserved as adjunctive therapy. Newer stereotactic computer assisted linear accelerator techniques (e.g., proton knife) may provide increased effectiveness with fewer side effects and be particularly useful in childhood Cushing's disease. Appropriate treatment of children with Cushing's disease can be compatible with satisfactory psychosocial adjustment and fertility in adulthood.