Endogenous Cushing's Syndrome (CS) is a relatively uncommon disease in children. The etiology is more often a primary adrenal cause in infants and younger children, and corticotroph adenoma in older ones. Diagnostic evaluation must be stepwise and methodical, aimed at first confirming the presence of the disease, and then looking for the etiology. The management should preferably be carried out at a centre which is experienced in the management of CS. Recent advances, including MRI, sensitive ACTH assays, inferior petrosal sinus sampling (IPSS) and transsphenoidal surgery (TSS) have dramatically improved the care of children with CS. However, the issue of high recurrence rates after TSS in children remains to be tackled. The prognosis of adrenal carcinoma still remains, dismal.