Purpose  To report a girl who are highly suspicious of the diagnoses of Juvenile xanthogranuloma(JXG).
Method case report and literature review.
Result  a 7-year-old girl who developed a painless yellowish lesion at corneal limbus and the neighboring conjunctiva, were evaluated. We excised the tumor on the conjunctival aspect and performed biopsy on the corneal aspect. After operation, the patient was treated with local steroid therapy. Typical Touton giant cells were not found in pathological section and immunohistochemical staining was positive for CD68. No recurrence was observed at last follow-up.

Conclusion: Juvenile xanthogranuloma only affecting the corneoscleral limbus is a rare in clinical routine. Immunohistochemical assay has significant diagnostic value in those cases absent of typical Touton giant cells.