Abstract: This paper reports a rare case of systemic juvenile xanthogranuloma (SJXG) with laryngeal involvement as the initial manifestation, followed by multi-system lesions involving the eyes, skin, liver and spleen. The diagnosis was confirmed based on characteristic pathological morphology and immunohistochemical findings, and the patient achieved symptomatic improvement after chemotherapy with vindesine combined with prednisone.

Key words: systemic juvenile xanthogranuloma, immunohistochemistry, larynx