A case of mucosal leishmaniasis in a 60-year old hemodialysis patient who had never lived outside Italy is described. The patient complained of fever, epistaxis and nasal obstruction. An anterior rhinoscopy disclosed a mass of two centimetres in diameter in the right nasal fossa. Histological examination revealed Leishmania amastigotes. Serology for Leishmania was positive with antibody titer of 1/320. A culture yielded a very slow growth of Leishmania infantum MON-24. In spite of a two-month treatment with oral itraconazole, the lesions progressively worsened. Treatment with topical paromomycin sulfate determined the complete resolution of the lesions within four months, with a residual perforation of the septum. This case demonstrates that localization of Leishmania spp must be considered in the differential diagnosis of mucosal lesions in hemodialyzed patients, even in countries not at risk for this parasite. Moreover, this case indicates the important role of the immune system in the evolution of the disease.