Purpose: To report the clinical and histopathologic findings of limbal juvenile xanthogranuloma (JXG) in two children. Methods: Case report. Results: Two children (a 9-month old boy and a 4-year old girl) presented with an enlarging yellowish mass at the corneoscleral limbus. The girl had been diagnosed with neurofibromatosis 1 (NF1). Both lesions underwent simple excision. Histopathologic examination revealed foamy histiocytes within an inflammatory infiltrate with the presence of multiple multinucleated giant cells. In both cases intraoperative subconjunctival steroids and postoperative topical steroids were administered during 1 month. Conclusions: Juvenile xanthogranuloma may rarely present as a mass occurring at the corneoscleral limbus. Therefore it should be considered in the differential diagnosis of any corneoscleral limbal mass lesion, particularly in children. Most are isolated lesions without systemic involvement which are treated by simple excision. But association with NF1 is possible and in that case screening for hematological malignancies is recommended.