We report a case of juvenile xanthogranuloma associated with neurofibromatosis. The patient was a 10-month-old female who had multiple yellowish papules on the face and shoulders A Biopsy of these lesions revealed foamy histiocytes with an admixture of inflammatory cells and Touton giant cells. Multiple, more than 6, cafe-au-lait spots, measuring more than 1.5cm in diameter were scattered on the trunk and buttocks Retardation of physical growth and development was observed.