Benign juvenile xanthogranuloma is a normolipaemic, self-limiting condition usually presenting with cutaneous, orbital or occasionally with visceral lesions. It is one type of histiocytosis, a term referring to a group of non-inflammatory, proliferative disorders of the monocyte/macrophage and dendritic cell systems [7]. We report a case of benign juvenile xanthogranuloma occurring in the larynx, a location not previously described.