Background:Unilateral tumors or masses of the adrenal gland are common. They are categorized as either functional (hormone-secreting) or silent and as either benign or malignant. Adrenocortical tumors are rare in childhood, with an incidence of 0.3-0.5 cases per one million child-years. Almost half of childhood tumors are adrenocortical carcinomas (ACC). Most ACCs are sporadic, but specialy in children, some occur as a component of hereditary cancer syndromes. The most common presenting symptom occurring in 50-80% in children with ACC is virilization; overall, survival is poor for adrenocortical carcinoma (ACC). The prognosis in children who have adrenocortical carcinoma (ACC) appears to be better than that of adults, at least for early-stage disease. Residual or metastatic disease carries a poor prognosis. Complete resection is required for cure.

Case presentation: The patient is a 8 years old girl  presented progressive virilizing symptoms including  deepening of the voice, clitoromegaly , growth acceleration, acne and premature pubic and axillary hair development before 8 years old.

Conclusion: The clinical, biochemical, histological features along with differential diagnosis are discussed. This case is presented because of its rarity.This case study report a rare case and also to highlight the importance of differentiating ACC from an adenoma particularly in pediatric patients.