Abstract: A 6-month-old male infant with generalized juvenile xanthogranuloma was reported and related literature was reviewed. The infant presented with a 3-month history of brown-yellow papules scattered on the face，trunk and extremities. Skin biopsy showed a dermal infiltrate of many histiocytes, foam cell and Touton giant cell, eosinophilic granulocytes and a small number of lymphocytes. Immunohistochemical examination of the lesions showed positive staining for CD68, S-100 and negative for CD1a and langerin antibodie．Birbeck granules was not found under electron microscope.  The diagnosis of generalized juvenile xanthogranuloma was made.

Key words: generalized xanthogranuloma, S-100 protein