Although leishmaniasis is an endemic disease in Iran the mucosal involvement is rare. Mucocutaneous leishmaniasis (MCL) mainly caused by Leishmanial braziliensis infection, reported with other Leishmania species such as L. major. Herein a 78 yr old man with MCL from Iran is presented who referred to Razi Hospital Dermatology Clinic, Tehran, Iran, for multiple ulcerative lesions on mid face skin, mucosa of upper lip and anterior fossa of nose, dorsal aspect of the hands and the posterior aspect of heels. Skin biopsy revealed necrotizing and granulomatous tissue pattern that suggested infection pathogenesis but the smear for leishmaniasis, Mycobacterium spp, and fungal elements was negative at first. In order to a positive PPD test, he was treated by anti-tuberculosis treatment. A month after starting drugs for tuberculosis, the prepared microscopical smears were positive for Leishman bodies this time. The skin biopsy revealed amastigote forms of Leishmania sp. and the PCR assay on specimens of lesions proved L. major as the principal pathogenic agent. There was good response to systemic treatment with meglumine antimoniate (Glucantime®) 3 gr per day until one week followed by 4.5 gr per day for another week. We forced to discontinue of drug because of cardiac toxicity at the end of 2nd wk of treatment.