Background: Cushing syndrome (CS) in childhood is an extremely rare disorder that poses unique diagnostic difficulties. Different etiopathological states account for endogenous hypercortisolism in various age groups. Diagnosis is especially challenging in individuals with adrenocorticotropic hormone-dependent CS. The two most common etiologies are adrenal tumors and pituitary corticotrophin- secreting adenomas (Cushing disease). Case Report: We present the case of a 9-year-old boy that illustrates some of the challenges of diagnosing and managing patients with CS. Conclusions: Diagnosis is usually made by an experienced clinician using a combination of tests to establish a state of endogenous hypercortisolism followed by localization of its source.Management requires an individualized approach and multidisciplinary care. Even after curative therapy, these children require long-term follow-up into adulthood to assess growth and body composition and routine monitoring for metabolic anomalies and/or recurrence of the disease.