A cortical adrenal carcinoma arising during the neonatal period and associated with virilization recurred after an initial excision and is in complete remission after a follow-up period of 10 years. The authors have performed a review of the medical literature which has yielded 56 neonatal carcinomas including this one. More than half of these cases concerned the thyroid gland (15 cases) and the adrenal cortex (14 cases). These were followed by malignant melanomas (12 cases), carcinomas of the parotid gland (6 cases), oropharyngeal region (3 cases), adrenal medulla (2 cases) and stomach, liver, breast and cutaneous sweat gland (one case each). Six tumors were associated with congenital malformations which included Beckwith-Wiedeman's syndrome; 4 tumors arose from preexisting tissue lesions; in one case, oral contraceptives were taken during pregnancy. The main treatment of neonatal carcinomas is surgery. Local recurrences (7 cases) do not markedly affect the outcome, whereas metastases (12 cases) have a very unfavorable prognosis. Superficial tumors are diagnosed earlier and more frequently cured. Out of 44 cases for which the outcome is known, only 18 children survived.