Abstract
Congenital Adrenal Hyperplasia has been associated with an increased prevalence of adrenal masses. It is still unknown whether incidentalomas in CAH* patients are more frequent or if the risk of adrenal carcinoma is higher than the general population. Therefore, the management CAH subjects presenting with suspicious adrenal masses remains problematic. We relate the case of a patient with CAH presenting for an adrenal incidentaloma with malignant features. The management of such cases is controversial as surgery is risky for large masses. Despite dimensions, a laparoscopic approach was used for resection. The patient remained disease free at 4 years post-operatively.