Juvenile xanthogranulomas (JXGs) are uncommon non-Langerhans cell histiocytic proliferations which occur most often in children. Rare cases of intracranial JXGs in children have been reported. The precise treatment strategy for intracranial JXG with high fatality is still unclear. We present four cases of intracranial JXG with 2-6 years of follow-up. Review of the previous literature since 1980 revealed another 39 pediatric intracranial JXGs. Their clinical characteristics varied significantly. Most intracranial JXGs presented in young children (88 %). Males (72 %) were affected more often than females. The differential diagnosis included two important components: the magnetic resonance imaging (MRI) characteristics and the pathohistiocytic markers. Statistical analysis suggested that there were no significant association between resection of intracranial lesions, multiple intracranial lesions, systematic lesions and clinic outcome (p = 0.12, p = 0.13, p = 0.60 respectively). Also, the manifestation with multiple intracranial lesions did not have a significant association with systematic JXG (p = 0.26). We found no significant associations between clinic characteristics, surgical resection and outcome. When feasible, total surgical resection of intracranial lesion may be curative.
