We present a case of a 3-year-old male child with a history of swelling in the left eye of 1-month duration who was seen elsewhere for this complaint and was started on topical steroids. Slit-lamp examination revealed a conjunctival swelling of size 2 mm × 2 mm, well-defined margins, slightly raised from the surface, had a pink hue without any surrounding conjunctival congestion. A clinical diagnosis of foreign body granuloma was made, and the child underwent excision biopsy. Histopathological examination revealed Juvenile Xanthogranuloma (JXG). JXG was first described by Adamson in 1905 in a child who had multiple skin nodules. He defined those lesions by the name congenital xanthoma multiplex. A case of JXG with iris involvement was presented for the first time in 1948 at the Ophthalmic Pathology Club gathering in Washington DC, and it was published 1 year later by Blank et al.