Although adrenocortical carcinoma is seen infrequently in children in most countries, it is occurs with significant frequency in Southern Brazil. This has enabled a study of the pattern of this disease in the pediatric age group. Of 78 patients with adrenocortical carcinoma seen at two hospitals in Curitiba, Brazil, 55 were adequately documented, with minimum of 2 years' follow-up, and formed the basis of this paper. Ninety-five percent of the patients showed some degree of virilization, notably pubic and/or body hair, clitoris or penis enlargement, and adult voice. Cushing's syndrome was present in 73% of these cases. The prognosis is age related and is dependent on the resectability of the tumor. Whereas 82% of the children under the age of 2 years survived, the figure for those over the age of 2 years was only 29%. When total resection of the tumor was possible, the survival rate was 67%, with no survival when only partial excision was performed.