Children with Beckwith-Wiedemann syndrome (BWs) have an increased risk of developing embryonic tumours during the first few years of life. Predisposition to the development of tumours is closely related to molecular subtype and therefore the cancer screening programme should be individualized on the basis of the genotype. Among the most common tumours, those with adrenal origin even if they show benign characteristics should be evaluated to rule out the hypothesis of severe tumours such as neuroblastoma. The paper reports the case of a newborn with BWs who showed an adrenal mass shortly after birth. To evaluate the lesion, though there is little available literature on the subject, one of the few existing algorithm for the diagnosis and follow-up was used. In case of ultrasound detection of an adrenal mass the algorithm first considers age, size and type of consistency (cystic or solid).