Adrenocortical carcinoma is extremely rare in children. The majority of cases presented with a combination of clinical features of Cushing syndrome and hyperandrogenism. The authors report three cases of isolated androgen-producing adrenocortical carcinoma with different clinical presentations. The two cases had clinical manifestations of hyperandrogenism: one boy with isosexual pseudoprecocity and one girl with heterosexual pseudoprecocity, both of whom underwent complete tumor removal and were well after surgery. The third patient presented with a huge abdominal mass and weight loss. Local and distant metastases (both lungs) were detected at the time of diagnosis. The patient expired after 36 days of hospitalization. The pathological section in all three patients demonstrated highly pleomorphism, increased mitoses, and scattered areas of necrosis. All cases had high levels of 1 7-hydroxyprogesterone, dehydroepiandrosterone-sulphate, and testosterone.