Systemic juvenile xanthogranuloma is a rare disease in children. A 10-year-old boy who showed renal, pulmonary, and liver involvement is reported. He had pulmonary invasion, renal mass, and nodular liver lesions but no bone involvement. The diagnosis was confirmed by renal biopsy, which revealed foamy, lipid-laden macrophages with positive CD68, but negative CD1a and S-100. The patient was treated with pulse high-dose methylprednisolone (10 mg/kg/d for 3 d for 6 courses). On 1-year follow-up period after 6 courses therapy was resulted in remarkable regression in renal and liver lesions.