Juvenile xanthogranuloma (JX) is a benign growth of fibrohistiocytoma range that mainly affects children. The most frequent localization of the tumor is skin integument. Non-cutaneous forms of JX are found in 5-10% of cases; they are manifested as deep lesions of soft tissues, involvement of various organs (oropharynx, lungs, liver, spleen, pericardium, gastrointestinal tract, central nervous system, bone marrow) including the organ of vision. JX can develop in any part of the eye and its appendages. Taking into consideration how rare this pathology is, its diagnosis and treatment are complicated. The article reviews 2 clinical cases of JX observed by the authors.