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Cerebral edema resulting in elevated intracranial pressure is a well-known complication of galactosemia. Lumbar puncture was performed for the diagnosis of clinically suspected bacterial meningitis. Herniation of cerebral tissue through the foramen magnum is not a common problem in neonatal intensive care units because of the open fontanelle in infants. We present the case of a 3-week-old infant with galactosemia who presented with signs of cerebellar herniation after lumbar puncture.