To report an 11-year-old Chinese girl with juvenile xanthogranuloma of the limbus, occurring in isolation without dermatologic involvement, and to discuss the clinical and histologic features, pathogenesis, and treatment of juvenile xanthogranuloma.Case report and review of medical literature.A total excision and biopsy of the limbal lesion with lamellar graft was performed. Histologic examination showed a xanthogranuloma. At 12-month follow-up, there was no recurrence of tumor.The diagnosis of juvenile xanthogranuloma may be straightforward in cases that have concomitant cutaneous manifestations. However, in isolated ocular forms, histopathological examination is necessary. Unlike the more common iris lesions that are preferably treated nonsurgically, limbal lesions require surgical excision.