We report a case of leishmaniasis localized to the upper lip in a 77-year-old Italian man. The disease was characterized by a severe swelling which involved the right side of the upper lip. The swelling was slightly erythematous with crusts and scaling. Consistency was parenchymatous-hard. The lesion was asymptomatic. Histopathologic examination showed an inflammatory infiltrate consisting of lymphocytes, histiocytes, and plasma cells. Several Leishmania spp. amastigotes were observed in the cytoplasm of macrophages. Cultural examination on Novy-MacNeal-Nicolle medium was positive for Leishmania spp. Polymerase chain reaction was positive for Leishmania infantum. The patient was treated with IM N-methylglucamine antimonate, with complete remission in approximately 6 weeks. During follow-up of 9 months, no relapses were observed.