Glioneuronal heterotopias are rare malformations composed of differentiated derivates of neuroectodermal tissue that include disorganized neuronal and glial cells. They are typically located extracranially. The nasal cavity represents the most common location. Glioneuronal heterotopias have also been reported in the orbit, pharynx, middle ear, neck and thorax. Rarely, glioneuronal heterotopias may be located intracranially. We are aware of only 15 patients reported with intracranial extra-axial glioneuronal heterotopia (IEGH). We present the prenatal ultrasound and MRI as well as the matching postnatal CT, conventional MRI and diffusion tensor imaging findings of a newborn with an infratentorial IEGH accompanied with craniofacial anomalies.
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