RESUMO
Juvenile xanthogranuloma (JXG) is a benign, normolipaemic, self-healing condition and a type of histiocytosis that occurs most frequently in infants and children, although adults may also be affected. This condition usually presents with a solitary or multiple cutaneous lesions and occasionally with extracutaneous lesions, especially the eye, lung, liver, kidney and pericardium. Histologically, JXG represents an accumulation of histiocytes lacking Birbeck granules (non-Langerhans cell), which can be differentiated from the Langerhans cells by specific staining techniques. The lesions may be excised for diagnostic, cosmetic or symptomatic reasons. We report a case of juvenile xanthogranuloma that occurred in the nasal cavity and testis for the first time.