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Congenital intracranial tumors are rare and only account for 0.5 to 1.5% of all pediatric brain tumors. Teratoma is the most frequently encountered intracranial tumor at birth. Cephalomegaly and hydrocephalus are the usual clinical presentations. Advances in imaging techniques have improved diagnostic accuracy in congenital brain tumors. But the prognosis of a massive intracranial teratoma is always dismal. We report a congenital mixed malignant germ cell tumor in a male fetus at 27 weeks of gestation, with massive involvement of cerebrum and orbit. According to histological and immunohistochemical studies, the tumor was made up of predominantly immature teratoma combined with a yolk sac tumor. To our knowledge, such a combination has not been well documented in the literature.

Congenital mixed malignant germ cell tumor involving cerebrum and orbit