Juvenile xanthogranuloma (JXG) is a rare disease that is part of a spectrum of histiocytic dendritic cell disorders. The authors report an unusual case of a 6-week-old male who presented with seizures. Neuroimaging revealed disseminated intracranial disease involving the optic apparatus, basal ganglia, lateral ventricles, and brainstem. The patient did not have any cutaneous lesions or evidence of extracranial disease. The patient underwent open biopsy of a large right midbrain lesion; pathology was consistent with JXG. He underwent postoperative chemotherapy and is doing well 7 months after surgery with regression of the intracranial lesions. To the best of the authors' knowledge, this is the first report of a neonate with disseminated intracranial JXG without cutaneous stigmata.
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