The authors present a case of mucocutaneous Leishmaniasis of the nose in a patient originally from French Guiana and review the characteristics of this parasitic disease and its importance in the differential diagnosis of lethal midline granuloma. Beyond the exotic pathology of this case, the review of the literature indicates that with the present extension of concomitant leishmaniasis and Hids, authentic autochtonal leishmaniasis may be responsible for otolaryngologic manifestations. The increased prevalence of leishmaniasis associated with AIDS, as well as the presence of mucosal lesions, seems to be underestimated.