Background: Juvenile xanthogranuloma (JXG) is a disorder of histiocyte proliferation. Most cases present with a solitary cutaneous lesion. JXG with systemic involvement is rare with significant morbidity. Intracranial solitary JXG may be misdiagnosed before operation.
Methods: A 5-month-old boy showed an elevated anterior fontanel but no other abnormalities on admission. Brain MRI showed a large mass in the right parietal region.
Results: The tumor was removed with the encroached meninges. A JXG in the right parietal region was diagnosed pathologically.
Conclusion: Total excision of the tumor may be curative with a prerequisite of ensuring normal vital signs and nervous function.