Adrenocortical cancers in childhood are very rare tumors. They are categorized as functional (hormone-secreting) or silent and as either benign or malignant. They have a bimodal distribution. Although in most adults they are non-functional, in the pediatric age group they may present as hormonal active or as an active tumor presenting with either virilizing forms or Cushing's syndrome or both sometimes. In children, due to the rapid development of symptoms, they come to attention early. However, if not diagnosed and treated early, they can develop into serious medical conditions. We present here a 6-year-old girl complaining of voice changes (deepening), extremely overweight, excessive hair growth over her body, and clitoromegaly for one year. Abdominal ultrasound and computed tomography revealed a well-defined adrenal mass with a slightly heterogeneous appearance and heterogeneous-contrast enhancement containing some necrotic areas. The patient was discharged one week after unilateral right adrenalectomy in good condition, and oral medications were given along with high-dose corticosteroid medications, which were reduced gradually. All the symptoms disappeared 6 months after the operation.
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