Neonatal brain tumor is a rare clinical entity. The initial presentation in affected newborn infants is often subtle, nonspecific, and rarely includes focal neurologic signs. We report a 2-day-old male infant with congenital oligodendroglioma with initial manifestations of hyperbilirubinemia. Phototherapy was started immediately after admission. Because of a tense anterior fontanel and irritable crying, head ultrasonography, computed tomography, and magnetic resonance imaging were performed and revealed a heterogenous brain tumor in the left temporoparietal lobe. Craniotomy and tumor evacuation was performed, and WHO grade III anaplastic oligodendroglioma was confirmed by the pathologic studies. The patient was discharged 3 weeks later, and outpatient follow-up examination revealed a normally developed infant at 6 months of age. The cause of jaundice was thought to be secondary to tumor hemorrhage, which was not infrequent in neonatal brain tumor.
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