The most common brain tumor that was present or produced symptoms at birth was teratoma. In this series of congenital tumors, teratomas occurred over 5 times more frequently than the second most common type, astrocytoma. They were often immature because of primitive neural elements and, rarely, a component of mixed malignant germ cell tumors. Incidence between sexes was nearly identical among histologic types that were reported at least 10 times, except for choroid plexus papilloma, which exhibited a male predominance. Four of five meningiomas occurred in males and 4 of 5 sarcomas in females. The predilection of neonatal brain tumors for supratentorial locations was also observed in this series of tumors present at birth. Teratomas occurred above the tentorium almost exclusively and all craniopharyngiomas and 14 of 16 choroid plexus tumors occurred supratentorially. At birth, large head or tense fontanel was a presenting sign in at least 55% of patients. Neurologic symptoms as initial symptoms were comparatively rare. Teratomas and craniopharyngiomas are tumors believed to arise because of developmental defects and these were the tumors that most often occurred with other anomalies. Anomalies were usually located in the head, with cleft lip or palate being most frequent. Prognosis for patients with brain tumors at birth was very poor, usually because of the massive size of the tumor. However, if small and favorably located, tumors were resected successfully. The most favorable outcomes were with choroid plexus tumors where aggressive treatment led to disease-free survival.