Congenital intracranial tumors are very rare and represent 0.5 to 1.5% of all pediatric brain tumors. [1, 2, 3, 4] According to Sclhembach D et al [4] the most common type of these tumors is teratoma. Teratoma is located primarily in the supratentorial brain and midline structures such as the pineal gland, quadrigeminal plate, walls of the third ventricle, suprasellar region, and cerebellar vermis. Its location in the lateral ventricle is extremely rare. We report a case of immature intracranial teratoma in a 2-day-old female with macrocephaly and hydrocephalus diagnosed as a brain tumor by ultrasound examination and confirmed at autopsy. Case reportA healthy 26-year-old primigravida was followed for a normal pregnancy. with a normal ultrasound at the 20th week. At 32 weeks of gestation, ultrasound revealed massive macrocephaly with hydrocephalus due to a right lateral ventricular choroid plexus mass. A week later, another ultrasound confirmed the diagnosis and showed increased tumor growth. Due to macrocephaly, labor was induced at the 34th week with a palliative care plan for the infant at birth in agreement with the family. The fetus was delivered by cesarean section due to failure to induce labor after 48 hours. The newborn weighed 2790 g and suffered from massive macrocephaly, his head circumference was 42 cm. The fontanels were enlarged and the cranial sutures were split. Otherwise, the infant had a normal-appearing body. After giving birth, he breathes after a few seconds and cries openly after ten minutes. During the first day, he repeatedly experienced oxygen desaturation and died on the second day after cardiac arrest. An autopsy was performed. The cranial bones were extremely thin. A huge tower......middle of paper......extension. Teratology. 1985;32(2):163-9.16-Uken P, Sato Y, Smith W. MR findings of malignant intracranial teratoma in a neonate. Pediatrician Radiol 1986; 16:504-50517- Buetow PC, Smimiotopoulos JG, Done S. Congenital brain tumors: a review of 45 cases. AJR 1990; 155:587-59318- Ferreira J, Eviatar L, Schneider S. Grossman R... Prenatal diagnosis of intracranial teratoma: prolonged survival after resection of a malignant teratoma diagnosed prenatally by ultrasound. A case report and literature review. Pediatr Neurosurg 1993;19:84-88.19- Oi S, Tamaki N, Kondo T, Nakamura H, Kudo H, Suzuki H, Sasaki M, Matsumoto S, Ueda Y, Katayama K,, Massive congenital intracranial teratoma diagnosed in utero. Children's Nervous System 1990; 6:459-46120-. Richards SR. Ultrasound diagnosis of intracranial teratoma in utero: report of a case and review of the literature. J Reprod Med 1987;12:73-75.