An 18-year-old patient presented with a solitary limbal tumor on his left eye that had appeared and increased in size for 3 weeks. This mass did not display any extension into the anterior chamber on gonioscopy. Excisional biopsy by lamellar sclerokeratectomy was carried out and the lesion was sent for histologic examination. The pathologic diagnosis showed the characteristic picture of juvenile xanthogranuloma with numerous Touton giant cells. There was no recurrence after a 9-month follow-up. The diagnosis of isolated ocular juvenile xanthogranuloma may be difficult and histologic examination of ocular lesions is often necessary. When juvenile xanthogranuloma affects the uvea, treatment is medical. When it appears as a limbal mass, treatment is surgical. Juvenile xanthogranuloma should always be considered a differential diagnosis of a limbal mass, especially in children.