Two cases of congenital brain tumor present at birth are reported. One patient was a baby girl with a huge teratoma measuring 8 X 6 X 5 cm; she died on her fourth day of life. The other patient had a large cystic astrocytoma, which was successfully removed; the patient is alive and in good health at 2 years of age and has no sign of recurrence of the tumor. Each baby had a large head, which caused dystocia at delivery. In addition to these two cases, we reviewed 200 cases of brain tumors reported in the literature presenting or producing symptoms or found incidentally at autopsy within 2 months after birth. We analyzed these cases in terms of initial symptoms and signs, sex distribution, histologic types and sites of tumors, prognosis, associated anomalies, familial occurrence, and hemorrhage from the tumor. The results of these analyses are described in detail, and the etiology of neonatal brain tumors is discussed.