ABSTRACT Juvenile xanthogranuloma (JXG) is a relatively uncommon, benign histiocytic proliferative disorder. One should be aware of this entity in order to make a diagnosis with the possibility of visceral involvement as well as associated medical conditions related to it. The present case report describes a case of JXG in a two-year old boy who presented with a circumscribed papule located over the vestibule of the right nostril. The lesion was histologically diagnosed as a JXG after surgical excision of the mass