Juvenile xanthogranuloma is a benign, normolipaemic, self-healing condition and a type of histiocytosis that occurs most frequently in infants and children. This condition usually presents with solitary or multiple cutaneous lesions and occasionally with visceral lesions. We report a case of juvenile xanthogranuloma occurring in the nasal cavity. We believe this is the first report, in the English literature, of juvenile xanthogranuloma occurring in this site.