To report a rare case of juvenile xanthogranuloma in the lacrimal sac.

An 8-year-old female presented to our hospital with epiphora and palpable mass on the left medial canthal area. As a symptom, a sticky and bloody discharge through the punctum had begun on occasion 3 months previously. When pressure was applied on the left medial canthal area, a mucoid discharge through the punctum occurred, and a solid mass was palpable. The mass localized in the lacrimal sac and upper nasolacrimal duct was observed on orbit CT. No invasion into the surrounding tissue was present. The authors of the present study decided to perform endonasal dacryocystorhinostomy, and subsequently a skin-colored mass was found in the lacrimal sac. The histopathological and immunohistochemical diagnosis was histiocytic proliferation, compatible with juvenile xanthogranuloma. Six months later, the patient complained of epiphora recurrence with palpable mass on medial canthal area. The tumor recurrence was confirmed with enhanced orbit CT finding 14 months after the first surgery. A second surgery using endonasal dacryocystorhinostomy was performed to remove the mass.