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Adrenocortical cancers in childhood are very rare tumors. The tumors have varied presentation - either virilizing forms or presentation with Cushing′s syndrome, or both. In children, due to the rapid development of symptoms they come to attention early, however, if not diagnosed and treated early can have a downhill course. The last decade has seen the emergence of new diagnostic imaging modalities. There is also intense ongoing research in newer treatment modalities as these tumors can be unresectable or have a high recurrence rate.